Strain Name:

STOCK Hbatm1Paz Hbbtm1Tow Tg(HBA-HBBs)41Paz/J

Stock Number:

003342

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Availability:

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Use Restrictions Apply, see Terms of Use
These mice mimic the phenotype of humans with sickle cell anemia.

Description

Strain Information

Type Mutant Stock; Targeted Mutation; Transgenic;
Additional information on Genetically Engineered and Mutant Mice.
Visit our online Nomenclature tutorial.
Mating SystemSee Colony Maintenance under the Health & Care tab         (Female x Male)   01-MAR-06
Specieslaboratory mouse
GenerationN1F7G24 (10-DEC-13)
Generation Definitions
 
Donating InvestigatorDr. Chris Paszty,   Amgen, Inc.

Appearance
Expected coat color from matings is black and agouti.

Description
This strain was engineered so that it no longer expresses mouse Hba and Hbb, but does express human HBA and HBB. It mimics the genetic, hematologic and histopathologic features that are found in humans afflicted with sickle cell anemia, including irreversibly sickled red blood cells, anemia and multiorgan pathology. Typically, ~20% of sickling mutant mice die between weaning and 14 weeks.

Development
A transgenic construct containing human HBA1 (hemoglobin, alpha 1), HBG2 (hemoglobin, gamma G, fetal component), HBG1 (hemoglobin, gamma A, fetal component), HBD (hemoglobin, delta) and HBBS (hemoglobin, beta, sickle allele) genes and the locus control region were injected into fertilized FVB/N mouse eggs. Mice carrying the transgene were bred to mutant mice bearing targeted mutations in the endogenous mouse Hba and Hbb genes. The STOCK background of this strain is a mixture of FVB/N, 129, DBA/2, Black Swiss and >50% C57BL/6 genomes. It was backcrossed to C57BL/6J one generation after importation to The Jackson Laboratory.

Control Information

  Control
   See control note: Homozygous for Hbatm1Paz, Heterozygous for Hbbtm1Tow, Hemizygous for Tg(HBA-HBBs)41Paz (non-sickling females from the colony)
   000664 C57BL/6J (approximate)
 
  Considerations for Choosing Controls

Related Strains

Strains carrying   Hbatm1Paz allele
002616   B6.129S7-Hbatm1Paz/J
View Strains carrying   Hbatm1Paz     (1 strain)

Strains carrying other alleles of HBA1
021452   STOCK Hbbd3th Tg(LCR-HBA1,LCR-HBB*)1Tow/J
View Strains carrying other alleles of HBA1     (1 strain)

View Strains carrying other alleles of HBB     (4 strains)

Strains carrying other alleles of HBG1
013071   B6;129-Hbatm1(HBA)Tow Hbbtm2(HBG1,HBB*)Tow/Hbbtm3(HBG1,HBB)Tow/J
View Strains carrying other alleles of HBG1     (1 strain)

Strains carrying other alleles of Hba
001622   B6.Cg-Gpi1a Hbath-J
001175   B6.Cg-Hbap/J
000745   B6.SEC-Hbab/J
000191   B6.SM-Hbad/J
013071   B6;129-Hbatm1(HBA)Tow Hbbtm2(HBG1,HBB*)Tow/Hbbtm3(HBG1,HBB)Tow/J
000802   WB.Cg-Hbath-J/J
View Strains carrying other alleles of Hba     (6 strains)

View Strains carrying other alleles of Hbb     (12 strains)

Phenotype

Phenotype Information

View Related Disease (OMIM) Terms

Related Disease (OMIM) Terms provided by MGI
- Characteristics of this human disease are associated with transgenes and other mutation types in the mouse.
Priapism, Familial Idiopathic
Sickle Cell Anemia
- Potential model based on gene homology relationships. Phenotypic similarity to the human disease has not been tested.
Alpha-Thalassemia   (HBB)
Beta-Thalassemia   (HBB)
Beta-Thalassemia, Dominant Inclusion Body Type   (HBB)
Heinz Body Anemias   (HBB)
Hemoglobin--Beta Locus; HBB   (HBB)
Malaria, Susceptibility to   (HBB)
- Potential model based on transgenic expression of an ortholog of a human gene that is associated with this disease. Phenotypic similarity to the human disease has not been tested.
Cyanosis, Transient Neonatal; TNCY   (HBG2)
Hemoglobin H Disease; HBH   (HBA1)
Hemoglobin--Alpha Locus 1; HBA1   (HBA1)
View Mammalian Phenotype Terms

Mammalian Phenotype Terms provided by MGI
      assigned by genotype

Hbatm1Paz/Hbatm1Paz Hbbtm1Tow/Hbbtm1Tow Tg(HBA-HBBs)41Paz/0

        involves: 129 * Black Swiss * C57BL/6 * DBA/2 * FVB/N
  • reproductive system phenotype
  • abnormal penile erection
    • mice exhibit increased corpus cavernosal smooth muscle relaxation in response to nerve stimulation   (MGI Ref ID J:135978)
    • however, treatment with an Adora2b antagonist or PEG-ADA reduces priapic activity   (MGI Ref ID J:135978)
    • priapism
      • mice exhibit priapism that is associated with elevated levels of adenosine   (MGI Ref ID J:135978)
      • however, treatment with an Adora2b antagonist or PEG-ADA reduces priapic activity   (MGI Ref ID J:135978)

Hbatm1Paz/Hbatm1Paz Hbbtm1Tow/Hbbtm1Tow Tg(HBA-HBBs)41Paz/?

        involves: 129S2/SvPas * 129S7/SvEvBrd * Black Swiss * C57BL/6 * DBA/2* FVB/N
  • mortality/aging
  • partial neonatal lethality
    • many pups turn purple and die within a few hours of birth, however, mice that survive live to become adults (reaching at least 7 months of age)   (MGI Ref ID J:44161)
  • hematopoietic system phenotype
  • abnormal erythrocyte morphology
    • erythrocytes have increased dynamic rigidity   (MGI Ref ID J:44161)
    • abnormal hemoglobin
      • erythrocytes contain an excess of alpha globin chain synthesis   (MGI Ref ID J:44161)
      • decreased mean corpuscular hemoglobin concentration   (MGI Ref ID J:44161)
      • decreased mean corpuscular hemoglobin   (MGI Ref ID J:44161)
    • anisopoikilocytosis
      • sickle cell shapes are observed at a frequency of 5-10% in oxygenated blood   (MGI Ref ID J:44161)
    • decreased hematocrit
      • average hematocrit levels are 65% of control at 3-7 months of age   (MGI Ref ID J:44161)
    • decreased mean corpuscular volume   (MGI Ref ID J:44161)
  • abnormal erythrocyte osmotic lysis
    • erythrocytes have decreased osmotic fragility   (MGI Ref ID J:44161)
  • anemia   (MGI Ref ID J:44161)
  • increased spleen weight
    • weight is increased 13-fold as compared to control   (MGI Ref ID J:44161)
  • reticulocytosis
    • reticulocyte counts are elevated at 3-7 months of age   (MGI Ref ID J:44161)
  • spleen vascular congestion
    • congested sinusoidal channels are observed in spleen   (MGI Ref ID J:44161)
  • homeostasis/metabolism phenotype
  • hypoxia
    • cause of death in some newborn mice   (MGI Ref ID J:44161)
  • increased kidney iron level
    • iron deposits found in the tubular epithelium of the kidney   (MGI Ref ID J:44161)
  • increased liver iron level
    • iron deposits found in Kupffer cells of liver   (MGI Ref ID J:44161)
  • renal fibrosis   (MGI Ref ID J:44161)
  • cardiovascular system phenotype
  • abnormal kidney blood vessel morphology
    • kidney infarction   (MGI Ref ID J:44161)
  • abnormal liver vasculature morphology
    • liver infarction   (MGI Ref ID J:44161)
  • abnormal lung vasculature morphology
    • lung infarction   (MGI Ref ID J:44161)
  • increased heart weight
    • weight is increased 2-fold as compared to control   (MGI Ref ID J:44161)
  • spleen vascular congestion
    • congested sinusoidal channels are observed in spleen   (MGI Ref ID J:44161)
  • renal/urinary system phenotype
  • abnormal kidney blood vessel morphology
    • kidney infarction   (MGI Ref ID J:44161)
  • increased kidney iron level
    • iron deposits found in the tubular epithelium of the kidney   (MGI Ref ID J:44161)
  • increased kidney weight
    • weight is increased 2-fold as compared to control   (MGI Ref ID J:44161)
  • kidney atrophy   (MGI Ref ID J:44161)
  • kidney cysts   (MGI Ref ID J:44161)
  • renal fibrosis   (MGI Ref ID J:44161)
  • immune system phenotype
  • increased spleen weight
    • weight is increased 13-fold as compared to control   (MGI Ref ID J:44161)
  • spleen vascular congestion
    • congested sinusoidal channels are observed in spleen   (MGI Ref ID J:44161)
  • liver/biliary system phenotype
  • abnormal liver vasculature morphology
    • liver infarction   (MGI Ref ID J:44161)
  • increased liver iron level
    • iron deposits found in Kupffer cells of liver   (MGI Ref ID J:44161)
  • respiratory system phenotype
  • abnormal lung vasculature morphology
    • lung infarction   (MGI Ref ID J:44161)
View Research Applications

Research Applications
This mouse can be used to support research in many areas including:

Hematological Research
Sickle Cell Anemia

Hbatm1Paz related

Hematological Research
Hemoglobin Defects

Hbbtm1Tow related

Genes & Alleles

Gene & Allele Information provided by MGI

 
Allele Symbol Hbatm1Paz
Allele Name targeted mutation 1, Chris Paszty
Allele Type Targeted (Null/Knockout)
Common Name(s) Hba0; Hba1-2del;
Mutation Made ByDr. Chris Paszty,   Amgen, Inc.
Strain of Origin129S7/SvEvBrd-Hprt<+>
ES Cell Line NameAB1
ES Cell Line Strain129S7/SvEvBrd-Hprt<+>
Gene Symbol and Name Hba, hemoglobin alpha chain complex
Chromosome 11
Molecular Note Both of the adult hemoglobin genes, alpha 1 and alpha 2, and the region between them were deleted and replaced with a neomycin resistance cassette by homologous recombination. [MGI Ref ID J:28392]
 
Allele Symbol Hbbtm1Tow
Allele Name targeted mutation 1, Timothy Townes
Allele Type Targeted (Null/Knockout)
Common Name(s) Hbb0; Hbbth3; Hbbtm1Tmt;
Mutation Made By Timothy Townes,   University of Alabama School of Medicine
Strain of Origin129S2/SvPas
ES Cell Line NameD3
ES Cell Line Strain129S2/SvPas
Gene Symbol and Name Hbb, hemoglobin beta chain complex
Chromosome 7
Molecular Note A genomic fragment encompassing all of Hbb-b1 and a 5' portion of Hbb-b2 was replaced with a neo cassette inserted by homologous recombination. [MGI Ref ID J:29087]
 
Allele Symbol Tg(HBA-HBBs)41Paz
Allele Name transgene insertion 41, Chris Paszty
Allele Type Transgenic (Inserted expressed sequence)
Common Name(s) Tg(Hu-miniLCRalpha1GgammaAgammadeltabetaS); miniLCRalpha1GgammaAgammadeltabetaS;
Mutation Made ByDr. Chris Paszty,   Amgen, Inc.
Strain of OriginFVB/N
Expressed Gene HBG1, hemoglobin, gamma A, human
Expressed Gene HBG2, hemoglobin, gamma G, human
Expressed Gene HBA1, hemoglobin, alpha 1, human
Expressed Gene HBD, hemoglobin, delta, human
Expressed Gene HBB, hemoglobin, beta, human
Promoter HBB, hemoglobin, beta, human
Promoter HBA1, hemoglobin, alpha 1, human
General Note In conjunction with Hbatm1Paz and Hbbtm1Tmt, transgenic mice express exclusively human sickle hemoglobin. These mice do not express mouse Hba and Hbb, but do express human HBA and HBB. Although chronically anemic, most of these mice survive for 2 to 9 months and are fertile. A significant percentage of sickle cell mice do not survive to adulthood. These mice display the major genetic, hematologic and histopathologic features found in humans with sickle cell disease: irreversibly sickled red cells, anemia, and multiorgan pathology.
Molecular Note The transgene contains sequences encoding the human proteins HBA1 (hemoglobin, alpha 1), HBG2 (hemoglobin, gamma G, fetal component), HBG1 (hemoglobin, gamma A, fetal component), HBD (hemoglobin, delta), and HBB S (hemoglobin, beta, sickle allele), and the locus control region (LCR). The HBBS allele contains an A to T transversion mutation in the sixth codon of HBB which causes an amino acid change from Glu to Val. The transgene promoter is multiple: from alpha and beta globin humanloci. Transgenic mice express human alpha hemoglobin, gamma hemoglobin, and sickle cell hemoglobin. [MGI Ref ID J:44161]
 
 
 
 
 

Genotyping

Genotyping Information

Genotyping Protocols

Hbbtm1Tow-PROBE, End Point Analysis
Hbatm1Paz, Standard PCR
Hbbtm1Towalternate3, Separated PCR
Tg(HBA-HBBs)41Paz, QPCR
Tg(HBA-HBBs)41Paz, Standard PCR


Helpful Links

Genotyping resources and troubleshooting

References

References provided by MGI

Selected Reference(s)

Paszty C; Brion CM; Manci E; Witkowska HE; Stevens ME; Mohandas N; Rubin EM. 1997. Transgenic knockout mice with exclusively human sickle hemoglobin and sickle cell disease [see comments] Science 278(5339):876-8. [PubMed: 9346488]  [MGI Ref ID J:44161]

Additional References

Barinaga M. 1997. Mutant mice mimic human sickle cell anemia [news; comment] Science 278(5339):803-4. [PubMed: 9381190]  [MGI Ref ID J:44159]

Ciavatta DJ; Ryan TM; Farmer SC; Townes TM. 1995. Mouse model of human beta zero thalassemia: targeted deletion of the mouse beta maj- and beta min-globin genes in embryonic stem cells. Proc Natl Acad Sci U S A 92(20):9259-63. [PubMed: 7568113]  [MGI Ref ID J:29087]

Ryan TM; Ciavatta DJ; Townes TM. 1997. Knockout-transgenic mouse model of sickle cell disease [see comments] Science 278(5339):873-6. [PubMed: 9346487]  [MGI Ref ID J:44160]

Hbatm1Paz related

Al-Hasani K; Vadolas J; Knaupp AS; Wardan H; Voullaire L; Williamson R; Ioannou PA. 2005. A 191-kb genomic fragment containing the human alpha-globin locus can rescue alpha-thalassemic mice. Mamm Genome 16(11):847-53. [PubMed: 16284800]  [MGI Ref ID J:103573]

Archer DR; Stiles JK; Newman GW; Quarshie A; Hsu LL; Sayavongsa P; Perry J; Jackson EM; Hibbert JM. 2008. C-reactive protein and interleukin-6 are decreased in transgenic sickle cell mice fed a high protein diet. J Nutr 138(6):1148-52. [PubMed: 18492848]  [MGI Ref ID J:136372]

Banerjee T; Kuypers FA. 2004. Reactive oxygen species and phosphatidylserine externalization in murine sickle red cells. Br J Haematol 124(3):391-402. [PubMed: 14717789]  [MGI Ref ID J:88553]

Barinaga M. 1997. Mutant mice mimic human sickle cell anemia [news; comment] Science 278(5339):803-4. [PubMed: 9381190]  [MGI Ref ID J:44159]

Belcher JD; Mahaseth H; Welch TE; Otterbein LE; Hebbel RP; Vercellotti GM. 2006. Heme oxygenase-1 is a modulator of inflammation and vaso-occlusion in transgenic sickle mice. J Clin Invest 116(3):808-16. [PubMed: 16485041]  [MGI Ref ID J:106486]

Bivalacqua TJ; Musicki B; Hsu LL; Berkowitz DE; Champion HC; Burnett AL. 2013. Sildenafil citrate-restored eNOS and PDE5 regulation in sickle cell mouse penis prevents priapism via control of oxidative/nitrosative stress. PLoS One 8(7):e68028. [PubMed: 23844149]  [MGI Ref ID J:204301]

Champion HC; Bivalacqua TJ; Takimoto E; Kass DA; Burnett AL. 2005. Phosphodiesterase-5A dysregulation in penile erectile tissue is a mechanism of priapism. Proc Natl Acad Sci U S A 102(5):1661-6. [PubMed: 15668387]  [MGI Ref ID J:96103]

Chang J; Patton JT; Sarkar A; Ernst B; Magnani JL; Frenette PS. 2010. GMI-1070, a novel pan-selectin antagonist, reverses acute vascular occlusions in sickle cell mice. Blood 116(10):1779-86. [PubMed: 20508165]  [MGI Ref ID J:164535]

Chang JC; Ye L; Kan YW. 2006. Correction of the sickle cell mutation in embryonic stem cells. Proc Natl Acad Sci U S A 103(4):1036-40. [PubMed: 16407095]  [MGI Ref ID J:105707]

Chantrathammachart P; Mackman N; Sparkenbaugh E; Wang JG; Parise LV; Kirchhofer D; Key NS; Pawlinski R. 2012. Tissue factor promotes activation of coagulation and inflammation in a mouse model of sickle cell disease. Blood 120(3):636-46. [PubMed: 22661702]  [MGI Ref ID J:189104]

Chen G; Zhang D; Fuchs TA; Manwani D; Wagner DD; Frenette PS. 2014. Heme-induced neutrophil extracellular traps contribute to the pathogenesis of sickle cell disease. Blood 123(24):3818-27. [PubMed: 24620350]  [MGI Ref ID J:210886]

Dasgupta T; Fabry ME; Kaul DK. 2010. Antisickling property of fetal hemoglobin enhances nitric oxide bioavailability and ameliorates organ oxidative stress in transgenic-knockout sickle mice. Am J Physiol Regul Integr Comp Physiol 298(2):R394-402. [PubMed: 20007516]  [MGI Ref ID J:156179]

Dasgupta T; Hebbel RP; Kaul DK. 2006. Protective effect of arginine on oxidative stress in transgenic sickle mouse models. Free Radic Biol Med 41(12):1771-80. [PubMed: 17157180]  [MGI Ref ID J:116710]

Fabry ME; Suzuka SM; Weinberg RS; Lawrence C; Factor SM; Gilman JG; Costantini F; Nagel RL. 2001. Second generation knockout sickle mice: the effect of HbF. Blood 97(2):410-8. [PubMed: 11154217]  [MGI Ref ID J:66961]

Gavins FN; Russell J; Senchenkova EL; De Almeida Paula L; Damazo AS; Esmon CT; Kirchhofer D; Hebbel RP; Granger DN. 2011. Mechanisms of enhanced thrombus formation in cerebral microvessels of mice expressing hemoglobin-S. Blood 117(15):4125-33. [PubMed: 21304105]  [MGI Ref ID J:172840]

Gutsaeva DR; Montero-Huerta P; Parkerson JB; Yerigenahally SD; Ikuta T; Head CA. 2014. Molecular mechanisms underlying synergistic adhesion of sickle red blood cells by hypoxia and low nitric oxide bioavailability. Blood 123(12):1917-26. [PubMed: 24429338]  [MGI Ref ID J:209498]

Gutsaeva DR; Parkerson JB; Yerigenahally SD; Kurz JC; Schaub RG; Ikuta T; Head CA. 2011. Inhibition of cell adhesion by anti-P-selectin aptamer: a new potential therapeutic agent for sickle cell disease. Blood 117(2):727-35. [PubMed: 20926770]  [MGI Ref ID J:168427]

Hanson MS; Xu H; Flewelen TC; Holzhauer SL; Retherford D; Jones DW; Frei AC; Pritchard KA Jr; Hillery CA; Hogg N; Wandersee NJ. 2013. A novel hemoglobin-binding peptide reduces cell-free hemoglobin in murine hemolytic anemia. Am J Physiol Heart Circ Physiol 304(2):H328-36. [PubMed: 23125208]  [MGI Ref ID J:192837]

Hebbel RP; Vercellotti GM; Pace BS; Solovey AN; Kollander R; Abanonu CF; Nguyen J; Vineyard JV; Belcher JD; Abdulla F; Osifuye S; Eaton JW; Kelm RJ Jr; Slungaard A. 2010. The HDAC inhibitors trichostatin A and suberoylanilide hydroxamic acid exhibit multiple modalities of benefit for the vascular pathobiology of sickle transgenic mice. Blood 115(12):2483-90. [PubMed: 20053759]  [MGI Ref ID J:159262]

Hidalgo A; Chang J; Jang JE; Peired AJ; Chiang EY; Frenette PS. 2009. Heterotypic interactions enabled by polarized neutrophil microdomains mediate thromboinflammatory injury. Nat Med 15(4):384-91. [PubMed: 19305412]  [MGI Ref ID J:149370]

Hsu LL; Champion HC; Campbell-Lee SA; Bivalacqua TJ; Manci EA; Diwan BA; Schimel DM; Cochard AE; Wang X; Schechter AN; Noguchi CT; Gladwin MT. 2007. Hemolysis in sickle cell mice causes pulmonary hypertension due to global impairment in nitric oxide bioavailability. Blood 109(7):3088-98. [PubMed: 17158223]  [MGI Ref ID J:145347]

Ieremia J; Blau CA. 2002. Limitations of a mouse model of sickle cell anemia. Blood Cells Mol Dis 28(2):146-51. [PubMed: 12064910]  [MGI Ref ID J:128113]

Kaul DK; Fabry ME; Suzuka SM; Zhang X. 2013. Antisickling fetal hemoglobin reduces hypoxia-inducible factor-1alpha expression in normoxic sickle mice: microvascular implications. Am J Physiol Heart Circ Physiol 304(1):H42-50. [PubMed: 23125209]  [MGI Ref ID J:192836]

Kaul DK; Liu XD; Chang HY; Nagel RL; Fabry ME. 2004. Effect of fetal hemoglobin on microvascular regulation in sickle transgenic-knockout mice. J Clin Invest 114(8):1136-45. [PubMed: 15489961]  [MGI Ref ID J:93424]

Kiefmann R; Rifkind JM; Nagababu E; Bhattacharya J. 2008. Red blood cells induce hypoxic lung inflammation. Blood 111(10):5205-14. [PubMed: 18270324]  [MGI Ref ID J:135569]

Kohli DR; Li Y; Khasabov SG; Gupta P; Kehl LJ; Ericson ME; Nguyen J; Gupta V; Hebbel RP; Simone DA; Gupta K. 2010. Pain-related behaviors and neurochemical alterations in mice expressing sickle hemoglobin: modulation by cannabinoids. Blood 116(3):456-65. [PubMed: 20304807]  [MGI Ref ID J:162824]

Lagoda G; Sezen SF; Hurt KJ; Cabrini MR; Mohanty DK; Burnett AL. 2014. Sustained nitric oxide (NO)-releasing compound reverses dysregulated NO signal transduction in priapism. FASEB J 28(1):76-84. [PubMed: 24076963]  [MGI Ref ID J:206623]

Lebensburger JD; Howard T; Hu Y; Pestina TI; Gao G; Johnson M; Zakharenko SS; Ware RE; Tuomanen EI; Persons DA; Rosch JW. 2012. Hydroxyurea therapy of a murine model of sickle cell anemia inhibits the progression of pneumococcal disease by down-modulating E-selectin. Blood 119(8):1915-21. [PubMed: 22130804]  [MGI Ref ID J:181749]

Levasseur DN; Ryan TM; Pawlik KM; Townes TM. 2003. Correction of a mouse model of sickle cell disease: lentiviral/antisickling beta-globin gene transduction of unmobilized, purified hematopoietic stem cells. Blood 102(13):4312-9. [PubMed: 12933581]  [MGI Ref ID J:134982]

Li J; Kim K; Hahm E; Molokie R; Hay N; Gordeuk VR; Du X; Cho J. 2014. Neutrophil AKT2 regulates heterotypic cell-cell interactions during vascular inflammation. J Clin Invest 124(4):1483-96. [PubMed: 24642468]  [MGI Ref ID J:209590]

Liu S; McConnell SC; Ryan TM. 2013. Erythropoiesis in the absence of adult hemoglobin. Mol Cell Biol 33(11):2241-51. [PubMed: 23530053]  [MGI Ref ID J:204029]

Manci EA; Hillery CA; Bodian CA; Zhang ZG; Lutty GA; Coller BS. 2006. Pathology of Berkeley sickle cell mice: similarities and differences with human sickle cell disease. Blood 107(4):1651-8. [PubMed: 16166585]  [MGI Ref ID J:129356]

Meiler SE; Wade M; Kutlar F; Yerigenahally SD; Xue Y; Moutouh-de Parseval LA; Corral LG; Swerdlow PS; Kutlar A. 2011. Pomalidomide augments fetal hemoglobin production without the myelosuppressive effects of hydroxyurea in transgenic sickle cell mice. Blood 118(4):1109-12. [PubMed: 21536862]  [MGI Ref ID J:174901]

Mi T; Abbasi S; Zhang H; Uray K; Chunn JL; Xia LW; Molina JG; Weisbrodt NW; Kellems RE; Blackburn MR; Xia Y. 2008. Excess adenosine in murine penile erectile tissues contributes to priapism via A2B adenosine receptor signaling. J Clin Invest 118(4):1491-501. [PubMed: 18340377]  [MGI Ref ID J:135978]

Noguchi CT; Gladwin M; Diwan B; Merciris P; Smith R; Yu X; Buzard G; Fitzhugh A; Keefer LK; Schechter AN; Mohandas N. 2001. Pathophysiology of a sickle cell trait mouse model: human alpha(beta)(S) transgenes with one mouse beta-globin allele. Blood Cells Mol Dis 27(6):971-7. [PubMed: 11831863]  [MGI Ref ID J:128124]

Paszty C; Mohandas N; Stevens ME; Loring JF; Liebhaber SA; Brion CM; Rubin EM. 1995. Lethal alpha-thalassaemia created by gene targeting in mice and its genetic rescue. Nat Genet 11(1):33-9. [PubMed: 7550311]  [MGI Ref ID J:28392]

Prado GN; Romero JR; Rivera A. 2013. Endothelin-1 receptor antagonists regulate cell surface-associated protein disulfide isomerase in sickle cell disease. FASEB J 27(11):4619-29. [PubMed: 23913858]  [MGI Ref ID J:203687]

Pritchard KA Jr; Feroah TR; Nandedkar SD; Holzhauer SL; Hutchins W; Schulte ML; Strunk RC; Debaun MR; Hillery CA. 2012. Effects of experimental asthma on inflammation and lung mechanics in sickle cell mice. Am J Respir Cell Mol Biol 46(3):389-96. [PubMed: 22033263]  [MGI Ref ID J:194579]

Pritchard KA Jr; Ou J; Ou Z; Shi Y; Franciosi JP; Signorino P; Kaul S; Ackland-Berglund C; Witte K; Holzhauer S; Mohandas N; Guice KS; Oldham KT; Hillery CA. 2004. Hypoxia-induced acute lung injury in murine models of sickle cell disease. Am J Physiol Lung Cell Mol Physiol 286(4):L705-14. [PubMed: 12972407]  [MGI Ref ID J:134696]

Romero JR; Suzuka SM; Nagel RL; Fabry ME. 2004. Expression of HbC and HbS, but not HbA, results in activation of K-Cl cotransport activity in transgenic mouse red cells. Blood 103(6):2384-90. [PubMed: 14615383]  [MGI Ref ID J:88563]

Russell JE; Liebhaber SA. 1998. Reversal of lethal alpha- and beta-thalassemias in mice by expression of human embryonic globins. Blood 92(9):3057-63. [PubMed: 9787139]  [MGI Ref ID J:114200]

Ryan TM; Ciavatta DJ; Townes TM. 1997. Knockout-transgenic mouse model of sickle cell disease [see comments] Science 278(5339):873-6. [PubMed: 9346487]  [MGI Ref ID J:44160]

Solovey A; Kollander R; Shet A; Milbauer LC; Choong S; Panoskaltsis-Mortari A; Blazar BR; Kelm RJ Jr; Hebbel RP. 2004. Endothelial cell expression of tissue factor in sickle mice is augmented by hypoxia/reoxygenation and inhibited by lovastatin. Blood 104(3):840-6. [PubMed: 15073034]  [MGI Ref ID J:92287]

Sparkenbaugh EM; Chantrathammachart P; Mickelson J; van Ryn J; Hebbel RP; Monroe DM; Mackman N; Key NS; Pawlinski R. 2014. Differential contribution of FXa and thrombin to vascular inflammation in a mouse model of sickle cell disease. Blood 123(11):1747-56. [PubMed: 24449213]  [MGI Ref ID J:209655]

Sundaram N; Tailor A; Mendelsohn L; Wansapura J; Wang X; Higashimoto T; Pauciulo MW; Gottliebson W; Kalra VK; Nichols WC; Kato GJ; Malik P. 2010. High levels of placenta growth factor in sickle cell disease promote pulmonary hypertension. Blood 116(1):109-12. [PubMed: 20335221]  [MGI Ref ID J:162823]

Vincent L; Vang D; Nguyen J; Gupta M; Luk K; Ericson ME; Simone DA; Gupta K. 2013. Mast cell activation contributes to sickle cell pathobiology and pain in mice. Blood 122(11):1853-62. [PubMed: 23775718]  [MGI Ref ID J:202290]

Voon HP; Wardan H; Vadolas J. 2007. Co-inheritance of alpha- and beta-thalassaemia in mice ameliorates thalassaemic phenotype. Blood Cells Mol Dis 39(2):184-8. [PubMed: 17493845]  [MGI Ref ID J:141719]

Wallace HA; Marques-Kranc F; Richardson M; Luna-Crespo F; Sharpe JA; Hughes J; Wood WG; Higgs DR; Smith AJ. 2007. Manipulating the mouse genome to engineer precise functional syntenic replacements with human sequence. Cell 128(1):197-209. [PubMed: 17218265]  [MGI Ref ID J:117887]

Wen J; Jiang X; Dai Y; Zhang Y; Tang Y; Sun H; Mi T; Phatarpekar PV; Kellems RE; Blackburn MR; Xia Y. 2010. Increased adenosine contributes to penile fibrosis, a dangerous feature of priapism, via A2B adenosine receptor signaling. FASEB J 24(3):740-9. [PubMed: 19858092]  [MGI Ref ID J:158034]

Whitney JB; Russell ES. 1978. New mutants and biochemical variants: Alpha thalassemia Mouse News Lett 58:47-48.  [MGI Ref ID J:45721]

Wood KC; Hebbel RP; Granger DN. 2005. Endothelial cell NADPH oxidase mediates the cerebral microvascular dysfunction in sickle cell transgenic mice. FASEB J 19(8):989-91. [PubMed: 15923406]  [MGI Ref ID J:128246]

Wood KC; Hebbel RP; Granger DN. 2004. Endothelial cell P-selectin mediates a proinflammatory and prothrombogenic phenotype in cerebral venules of sickle cell transgenic mice. Am J Physiol Heart Circ Physiol 286(5):H1608-14. [PubMed: 14704223]  [MGI Ref ID J:95603]

Xu J; Peng C; Sankaran VG; Shao Z; Esrick EB; Chong BG; Ippolito GC; Fujiwara Y; Ebert BL; Tucker PW; Orkin SH. 2011. Correction of sickle cell disease in adult mice by interference with fetal hemoglobin silencing. Science 334(6058):993-6. [PubMed: 21998251]  [MGI Ref ID J:177861]

Zhang Y; Dai Y; Wen J; Zhang W; Grenz A; Sun H; Tao L; Lu G; Alexander DC; Milburn MV; Carter-Dawson L; Lewis DE; Zhang W; Eltzschig HK; Kellems RE; Blackburn MR; Juneja HS; Xia Y. 2011. Detrimental effects of adenosine signaling in sickle cell disease. Nat Med 17(1):79-86. [PubMed: 21170046]  [MGI Ref ID J:168473]

de Jong K; Emerson RK; Butler J; Bastacky J; Mohandas N; Kuypers FA. 2001. Short survival of phosphatidylserine-exposing red blood cells in murine sickle cell anemia. Blood 98(5):1577-84. [PubMed: 11520810]  [MGI Ref ID J:131515]

Hbbtm1Tow related

Abdulmalik O; Safo MK; Chen Q; Yang J; Brugnara C; Ohene-Frempong K; Abraham DJ; Asakura T. 2005. 5-hydroxymethyl-2-furfural modifies intracellular sickle haemoglobin and inhibits sickling of red blood cells. Br J Haematol 128(4):552-61. [PubMed: 15686467]  [MGI Ref ID J:96026]

Archer DR; Stiles JK; Newman GW; Quarshie A; Hsu LL; Sayavongsa P; Perry J; Jackson EM; Hibbert JM. 2008. C-reactive protein and interleukin-6 are decreased in transgenic sickle cell mice fed a high protein diet. J Nutr 138(6):1148-52. [PubMed: 18492848]  [MGI Ref ID J:136372]

Banerjee T; Kuypers FA. 2004. Reactive oxygen species and phosphatidylserine externalization in murine sickle red cells. Br J Haematol 124(3):391-402. [PubMed: 14717789]  [MGI Ref ID J:88553]

Barinaga M. 1997. Mutant mice mimic human sickle cell anemia [news; comment] Science 278(5339):803-4. [PubMed: 9381190]  [MGI Ref ID J:44159]

Belcher JD; Mahaseth H; Welch TE; Otterbein LE; Hebbel RP; Vercellotti GM. 2006. Heme oxygenase-1 is a modulator of inflammation and vaso-occlusion in transgenic sickle mice. J Clin Invest 116(3):808-16. [PubMed: 16485041]  [MGI Ref ID J:106486]

Bivalacqua TJ; Musicki B; Hsu LL; Berkowitz DE; Champion HC; Burnett AL. 2013. Sildenafil citrate-restored eNOS and PDE5 regulation in sickle cell mouse penis prevents priapism via control of oxidative/nitrosative stress. PLoS One 8(7):e68028. [PubMed: 23844149]  [MGI Ref ID J:204301]

Champion HC; Bivalacqua TJ; Takimoto E; Kass DA; Burnett AL. 2005. Phosphodiesterase-5A dysregulation in penile erectile tissue is a mechanism of priapism. Proc Natl Acad Sci U S A 102(5):1661-6. [PubMed: 15668387]  [MGI Ref ID J:96103]

Chang J; Patton JT; Sarkar A; Ernst B; Magnani JL; Frenette PS. 2010. GMI-1070, a novel pan-selectin antagonist, reverses acute vascular occlusions in sickle cell mice. Blood 116(10):1779-86. [PubMed: 20508165]  [MGI Ref ID J:164535]

Chang JC; Ye L; Kan YW. 2006. Correction of the sickle cell mutation in embryonic stem cells. Proc Natl Acad Sci U S A 103(4):1036-40. [PubMed: 16407095]  [MGI Ref ID J:105707]

Chantrathammachart P; Mackman N; Sparkenbaugh E; Wang JG; Parise LV; Kirchhofer D; Key NS; Pawlinski R. 2012. Tissue factor promotes activation of coagulation and inflammation in a mouse model of sickle cell disease. Blood 120(3):636-46. [PubMed: 22661702]  [MGI Ref ID J:189104]

Chen G; Zhang D; Fuchs TA; Manwani D; Wagner DD; Frenette PS. 2014. Heme-induced neutrophil extracellular traps contribute to the pathogenesis of sickle cell disease. Blood 123(24):3818-27. [PubMed: 24620350]  [MGI Ref ID J:210886]

Ciavatta DJ; Ryan TM; Farmer SC; Townes TM. 1995. Mouse model of human beta zero thalassemia: targeted deletion of the mouse beta maj- and beta min-globin genes in embryonic stem cells. Proc Natl Acad Sci U S A 92(20):9259-63. [PubMed: 7568113]  [MGI Ref ID J:29087]

Dasgupta T; Fabry ME; Kaul DK. 2010. Antisickling property of fetal hemoglobin enhances nitric oxide bioavailability and ameliorates organ oxidative stress in transgenic-knockout sickle mice. Am J Physiol Regul Integr Comp Physiol 298(2):R394-402. [PubMed: 20007516]  [MGI Ref ID J:156179]

Dasgupta T; Hebbel RP; Kaul DK. 2006. Protective effect of arginine on oxidative stress in transgenic sickle mouse models. Free Radic Biol Med 41(12):1771-80. [PubMed: 17157180]  [MGI Ref ID J:116710]

Gavins FN; Russell J; Senchenkova EL; De Almeida Paula L; Damazo AS; Esmon CT; Kirchhofer D; Hebbel RP; Granger DN. 2011. Mechanisms of enhanced thrombus formation in cerebral microvessels of mice expressing hemoglobin-S. Blood 117(15):4125-33. [PubMed: 21304105]  [MGI Ref ID J:172840]

Gutsaeva DR; Parkerson JB; Yerigenahally SD; Kurz JC; Schaub RG; Ikuta T; Head CA. 2011. Inhibition of cell adhesion by anti-P-selectin aptamer: a new potential therapeutic agent for sickle cell disease. Blood 117(2):727-35. [PubMed: 20926770]  [MGI Ref ID J:168427]

Hanson MS; Xu H; Flewelen TC; Holzhauer SL; Retherford D; Jones DW; Frei AC; Pritchard KA Jr; Hillery CA; Hogg N; Wandersee NJ. 2013. A novel hemoglobin-binding peptide reduces cell-free hemoglobin in murine hemolytic anemia. Am J Physiol Heart Circ Physiol 304(2):H328-36. [PubMed: 23125208]  [MGI Ref ID J:192837]

Hebbel RP; Vercellotti GM; Pace BS; Solovey AN; Kollander R; Abanonu CF; Nguyen J; Vineyard JV; Belcher JD; Abdulla F; Osifuye S; Eaton JW; Kelm RJ Jr; Slungaard A. 2010. The HDAC inhibitors trichostatin A and suberoylanilide hydroxamic acid exhibit multiple modalities of benefit for the vascular pathobiology of sickle transgenic mice. Blood 115(12):2483-90. [PubMed: 20053759]  [MGI Ref ID J:159262]

Hidalgo A; Chang J; Jang JE; Peired AJ; Chiang EY; Frenette PS. 2009. Heterotypic interactions enabled by polarized neutrophil microdomains mediate thromboinflammatory injury. Nat Med 15(4):384-91. [PubMed: 19305412]  [MGI Ref ID J:149370]

Hsu LL; Champion HC; Campbell-Lee SA; Bivalacqua TJ; Manci EA; Diwan BA; Schimel DM; Cochard AE; Wang X; Schechter AN; Noguchi CT; Gladwin MT. 2007. Hemolysis in sickle cell mice causes pulmonary hypertension due to global impairment in nitric oxide bioavailability. Blood 109(7):3088-98. [PubMed: 17158223]  [MGI Ref ID J:145347]

Ieremia J; Blau CA. 2002. Limitations of a mouse model of sickle cell anemia. Blood Cells Mol Dis 28(2):146-51. [PubMed: 12064910]  [MGI Ref ID J:128113]

Kaul DK; Fabry ME; Suzuka SM; Zhang X. 2013. Antisickling fetal hemoglobin reduces hypoxia-inducible factor-1alpha expression in normoxic sickle mice: microvascular implications. Am J Physiol Heart Circ Physiol 304(1):H42-50. [PubMed: 23125209]  [MGI Ref ID J:192836]

Kaul DK; Liu XD; Chang HY; Nagel RL; Fabry ME. 2004. Effect of fetal hemoglobin on microvascular regulation in sickle transgenic-knockout mice. J Clin Invest 114(8):1136-45. [PubMed: 15489961]  [MGI Ref ID J:93424]

Kiefmann R; Rifkind JM; Nagababu E; Bhattacharya J. 2008. Red blood cells induce hypoxic lung inflammation. Blood 111(10):5205-14. [PubMed: 18270324]  [MGI Ref ID J:135569]

Kohli DR; Li Y; Khasabov SG; Gupta P; Kehl LJ; Ericson ME; Nguyen J; Gupta V; Hebbel RP; Simone DA; Gupta K. 2010. Pain-related behaviors and neurochemical alterations in mice expressing sickle hemoglobin: modulation by cannabinoids. Blood 116(3):456-65. [PubMed: 20304807]  [MGI Ref ID J:162824]

Lagoda G; Sezen SF; Hurt KJ; Cabrini MR; Mohanty DK; Burnett AL. 2014. Sustained nitric oxide (NO)-releasing compound reverses dysregulated NO signal transduction in priapism. FASEB J 28(1):76-84. [PubMed: 24076963]  [MGI Ref ID J:206623]

Lebensburger JD; Howard T; Hu Y; Pestina TI; Gao G; Johnson M; Zakharenko SS; Ware RE; Tuomanen EI; Persons DA; Rosch JW. 2012. Hydroxyurea therapy of a murine model of sickle cell anemia inhibits the progression of pneumococcal disease by down-modulating E-selectin. Blood 119(8):1915-21. [PubMed: 22130804]  [MGI Ref ID J:181749]

Levasseur DN; Ryan TM; Pawlik KM; Townes TM. 2003. Correction of a mouse model of sickle cell disease: lentiviral/antisickling beta-globin gene transduction of unmobilized, purified hematopoietic stem cells. Blood 102(13):4312-9. [PubMed: 12933581]  [MGI Ref ID J:134982]

Li J; Kim K; Hahm E; Molokie R; Hay N; Gordeuk VR; Du X; Cho J. 2014. Neutrophil AKT2 regulates heterotypic cell-cell interactions during vascular inflammation. J Clin Invest 124(4):1483-96. [PubMed: 24642468]  [MGI Ref ID J:209590]

Liu S; McConnell SC; Ryan TM. 2013. Erythropoiesis in the absence of adult hemoglobin. Mol Cell Biol 33(11):2241-51. [PubMed: 23530053]  [MGI Ref ID J:204029]

Manci EA; Hillery CA; Bodian CA; Zhang ZG; Lutty GA; Coller BS. 2006. Pathology of Berkeley sickle cell mice: similarities and differences with human sickle cell disease. Blood 107(4):1651-8. [PubMed: 16166585]  [MGI Ref ID J:129356]

McConnell SC; Huo Y; Liu S; Ryan TM. 2011. Human globin knock-in mice complete fetal-to-adult hemoglobin switching in postnatal development. Mol Cell Biol 31(4):876-83. [PubMed: 21173165]  [MGI Ref ID J:170380]

Meiler SE; Wade M; Kutlar F; Yerigenahally SD; Xue Y; Moutouh-de Parseval LA; Corral LG; Swerdlow PS; Kutlar A. 2011. Pomalidomide augments fetal hemoglobin production without the myelosuppressive effects of hydroxyurea in transgenic sickle cell mice. Blood 118(4):1109-12. [PubMed: 21536862]  [MGI Ref ID J:174901]

Mi T; Abbasi S; Zhang H; Uray K; Chunn JL; Xia LW; Molina JG; Weisbrodt NW; Kellems RE; Blackburn MR; Xia Y. 2008. Excess adenosine in murine penile erectile tissues contributes to priapism via A2B adenosine receptor signaling. J Clin Invest 118(4):1491-501. [PubMed: 18340377]  [MGI Ref ID J:135978]

Nandedkar SD; Feroah TR; Hutchins W; Weihrauch D; Konduri KS; Wang J; Strunk RC; DeBaun MR; Hillery CA; Pritchard KA. 2008. Histopathology of experimentally induced asthma in a murine model of sickle cell disease. Blood 112(6):2529-38. [PubMed: 18579795]  [MGI Ref ID J:138890]

Noguchi CT; Gladwin M; Diwan B; Merciris P; Smith R; Yu X; Buzard G; Fitzhugh A; Keefer LK; Schechter AN; Mohandas N. 2001. Pathophysiology of a sickle cell trait mouse model: human alpha(beta)(S) transgenes with one mouse beta-globin allele. Blood Cells Mol Dis 27(6):971-7. [PubMed: 11831863]  [MGI Ref ID J:128124]

Prado GN; Romero JR; Rivera A. 2013. Endothelin-1 receptor antagonists regulate cell surface-associated protein disulfide isomerase in sickle cell disease. FASEB J 27(11):4619-29. [PubMed: 23913858]  [MGI Ref ID J:203687]

Pritchard KA Jr; Feroah TR; Nandedkar SD; Holzhauer SL; Hutchins W; Schulte ML; Strunk RC; Debaun MR; Hillery CA. 2012. Effects of experimental asthma on inflammation and lung mechanics in sickle cell mice. Am J Respir Cell Mol Biol 46(3):389-96. [PubMed: 22033263]  [MGI Ref ID J:194579]

Pritchard KA Jr; Ou J; Ou Z; Shi Y; Franciosi JP; Signorino P; Kaul S; Ackland-Berglund C; Witte K; Holzhauer S; Mohandas N; Guice KS; Oldham KT; Hillery CA. 2004. Hypoxia-induced acute lung injury in murine models of sickle cell disease. Am J Physiol Lung Cell Mol Physiol 286(4):L705-14. [PubMed: 12972407]  [MGI Ref ID J:134696]

Ramos P; Casu C; Gardenghi S; Breda L; Crielaard BJ; Guy E; Marongiu MF; Gupta R; Levine RL; Abdel-Wahab O; Ebert BL; Van Rooijen N; Ghaffari S; Grady RW; Giardina PJ; Rivella S. 2013. Macrophages support pathological erythropoiesis in polycythemia vera and beta-thalassemia. Nat Med 19(4):437-45. [PubMed: 23502961]  [MGI Ref ID J:197988]

Romero JR; Suzuka SM; Nagel RL; Fabry ME. 2004. Expression of HbC and HbS, but not HbA, results in activation of K-Cl cotransport activity in transgenic mouse red cells. Blood 103(6):2384-90. [PubMed: 14615383]  [MGI Ref ID J:88563]

Ryan TM; Ciavatta DJ; Townes TM. 1997. Knockout-transgenic mouse model of sickle cell disease [see comments] Science 278(5339):873-6. [PubMed: 9346487]  [MGI Ref ID J:44160]

Solovey A; Kollander R; Shet A; Milbauer LC; Choong S; Panoskaltsis-Mortari A; Blazar BR; Kelm RJ Jr; Hebbel RP. 2004. Endothelial cell expression of tissue factor in sickle mice is augmented by hypoxia/reoxygenation and inhibited by lovastatin. Blood 104(3):840-6. [PubMed: 15073034]  [MGI Ref ID J:92287]

Sparkenbaugh EM; Chantrathammachart P; Mickelson J; van Ryn J; Hebbel RP; Monroe DM; Mackman N; Key NS; Pawlinski R. 2014. Differential contribution of FXa and thrombin to vascular inflammation in a mouse model of sickle cell disease. Blood 123(11):1747-56. [PubMed: 24449213]  [MGI Ref ID J:209655]

Sundaram N; Tailor A; Mendelsohn L; Wansapura J; Wang X; Higashimoto T; Pauciulo MW; Gottliebson W; Kalra VK; Nichols WC; Kato GJ; Malik P. 2010. High levels of placenta growth factor in sickle cell disease promote pulmonary hypertension. Blood 116(1):109-12. [PubMed: 20335221]  [MGI Ref ID J:162823]

Szuber N; Buss JL; Soe-Lin S; Felfly H; Trudel M; Ponka P. 2008. Alternative treatment paradigm for thalassemia using iron chelators. Exp Hematol 36(7):773-85. [PubMed: 18456387]  [MGI Ref ID J:136991]

Vincent L; Vang D; Nguyen J; Gupta M; Luk K; Ericson ME; Simone DA; Gupta K. 2013. Mast cell activation contributes to sickle cell pathobiology and pain in mice. Blood 122(11):1853-62. [PubMed: 23775718]  [MGI Ref ID J:202290]

Wen J; Jiang X; Dai Y; Zhang Y; Tang Y; Sun H; Mi T; Phatarpekar PV; Kellems RE; Blackburn MR; Xia Y. 2010. Increased adenosine contributes to penile fibrosis, a dangerous feature of priapism, via A2B adenosine receptor signaling. FASEB J 24(3):740-9. [PubMed: 19858092]  [MGI Ref ID J:158034]

Wood KC; Hebbel RP; Granger DN. 2005. Endothelial cell NADPH oxidase mediates the cerebral microvascular dysfunction in sickle cell transgenic mice. FASEB J 19(8):989-91. [PubMed: 15923406]  [MGI Ref ID J:128246]

Wood KC; Hebbel RP; Granger DN. 2004. Endothelial cell P-selectin mediates a proinflammatory and prothrombogenic phenotype in cerebral venules of sickle cell transgenic mice. Am J Physiol Heart Circ Physiol 286(5):H1608-14. [PubMed: 14704223]  [MGI Ref ID J:95603]

Xu J; Peng C; Sankaran VG; Shao Z; Esrick EB; Chong BG; Ippolito GC; Fujiwara Y; Ebert BL; Tucker PW; Orkin SH. 2011. Correction of sickle cell disease in adult mice by interference with fetal hemoglobin silencing. Science 334(6058):993-6. [PubMed: 21998251]  [MGI Ref ID J:177861]

Zhang Y; Dai Y; Wen J; Zhang W; Grenz A; Sun H; Tao L; Lu G; Alexander DC; Milburn MV; Carter-Dawson L; Lewis DE; Zhang W; Eltzschig HK; Kellems RE; Blackburn MR; Juneja HS; Xia Y. 2011. Detrimental effects of adenosine signaling in sickle cell disease. Nat Med 17(1):79-86. [PubMed: 21170046]  [MGI Ref ID J:168473]

de Jong K; Emerson RK; Butler J; Bastacky J; Mohandas N; Kuypers FA. 2001. Short survival of phosphatidylserine-exposing red blood cells in murine sickle cell anemia. Blood 98(5):1577-84. [PubMed: 11520810]  [MGI Ref ID J:131515]

Tg(HBA-HBBs)41Paz related

Archer DR; Stiles JK; Newman GW; Quarshie A; Hsu LL; Sayavongsa P; Perry J; Jackson EM; Hibbert JM. 2008. C-reactive protein and interleukin-6 are decreased in transgenic sickle cell mice fed a high protein diet. J Nutr 138(6):1148-52. [PubMed: 18492848]  [MGI Ref ID J:136372]

Barinaga M. 1997. Mutant mice mimic human sickle cell anemia [news; comment] Science 278(5339):803-4. [PubMed: 9381190]  [MGI Ref ID J:44159]

Belcher JD; Bryant CJ; Nguyen J; Bowlin PR; Kielbik MC; Bischof JC; Hebbel RP; Vercellotti GM. 2003. Transgenic sickle mice have vascular inflammation. Blood 101(10):3953-9. [PubMed: 12543857]  [MGI Ref ID J:83445]

Belcher JD; Mahaseth H; Welch TE; Otterbein LE; Hebbel RP; Vercellotti GM. 2006. Heme oxygenase-1 is a modulator of inflammation and vaso-occlusion in transgenic sickle mice. J Clin Invest 116(3):808-16. [PubMed: 16485041]  [MGI Ref ID J:106486]

Bivalacqua TJ; Musicki B; Hsu LL; Berkowitz DE; Champion HC; Burnett AL. 2013. Sildenafil citrate-restored eNOS and PDE5 regulation in sickle cell mouse penis prevents priapism via control of oxidative/nitrosative stress. PLoS One 8(7):e68028. [PubMed: 23844149]  [MGI Ref ID J:204301]

Champion HC; Bivalacqua TJ; Takimoto E; Kass DA; Burnett AL. 2005. Phosphodiesterase-5A dysregulation in penile erectile tissue is a mechanism of priapism. Proc Natl Acad Sci U S A 102(5):1661-6. [PubMed: 15668387]  [MGI Ref ID J:96103]

Chang J; Patton JT; Sarkar A; Ernst B; Magnani JL; Frenette PS. 2010. GMI-1070, a novel pan-selectin antagonist, reverses acute vascular occlusions in sickle cell mice. Blood 116(10):1779-86. [PubMed: 20508165]  [MGI Ref ID J:164535]

Chang JC; Ye L; Kan YW. 2006. Correction of the sickle cell mutation in embryonic stem cells. Proc Natl Acad Sci U S A 103(4):1036-40. [PubMed: 16407095]  [MGI Ref ID J:105707]

Chantrathammachart P; Mackman N; Sparkenbaugh E; Wang JG; Parise LV; Kirchhofer D; Key NS; Pawlinski R. 2012. Tissue factor promotes activation of coagulation and inflammation in a mouse model of sickle cell disease. Blood 120(3):636-46. [PubMed: 22661702]  [MGI Ref ID J:189104]

Chen G; Zhang D; Fuchs TA; Manwani D; Wagner DD; Frenette PS. 2014. Heme-induced neutrophil extracellular traps contribute to the pathogenesis of sickle cell disease. Blood 123(24):3818-27. [PubMed: 24620350]  [MGI Ref ID J:210886]

Dasgupta T; Fabry ME; Kaul DK. 2010. Antisickling property of fetal hemoglobin enhances nitric oxide bioavailability and ameliorates organ oxidative stress in transgenic-knockout sickle mice. Am J Physiol Regul Integr Comp Physiol 298(2):R394-402. [PubMed: 20007516]  [MGI Ref ID J:156179]

Dasgupta T; Hebbel RP; Kaul DK. 2006. Protective effect of arginine on oxidative stress in transgenic sickle mouse models. Free Radic Biol Med 41(12):1771-80. [PubMed: 17157180]  [MGI Ref ID J:116710]

Gavins FN; Russell J; Senchenkova EL; De Almeida Paula L; Damazo AS; Esmon CT; Kirchhofer D; Hebbel RP; Granger DN. 2011. Mechanisms of enhanced thrombus formation in cerebral microvessels of mice expressing hemoglobin-S. Blood 117(15):4125-33. [PubMed: 21304105]  [MGI Ref ID J:172840]

Gutsaeva DR; Montero-Huerta P; Parkerson JB; Yerigenahally SD; Ikuta T; Head CA. 2014. Molecular mechanisms underlying synergistic adhesion of sickle red blood cells by hypoxia and low nitric oxide bioavailability. Blood 123(12):1917-26. [PubMed: 24429338]  [MGI Ref ID J:209498]

Gutsaeva DR; Parkerson JB; Yerigenahally SD; Kurz JC; Schaub RG; Ikuta T; Head CA. 2011. Inhibition of cell adhesion by anti-P-selectin aptamer: a new potential therapeutic agent for sickle cell disease. Blood 117(2):727-35. [PubMed: 20926770]  [MGI Ref ID J:168427]

He Z; Russell JE. 2004. Antisickling effects of an endogenous human alpha-like globin. Nat Med 10(4):365-7. [PubMed: 15034572]  [MGI Ref ID J:131270]

Hidalgo A; Chang J; Jang JE; Peired AJ; Chiang EY; Frenette PS. 2009. Heterotypic interactions enabled by polarized neutrophil microdomains mediate thromboinflammatory injury. Nat Med 15(4):384-91. [PubMed: 19305412]  [MGI Ref ID J:149370]

Holtzclaw JD; Jack D; Aguayo SM; Eckman JR; Roman J; Hsu LL. 2004. Enhanced pulmonary and systemic response to endotoxin in transgenic sickle mice. Am J Respir Crit Care Med 169(6):687-95. [PubMed: 14684557]  [MGI Ref ID J:132062]

Hsu LL; Champion HC; Campbell-Lee SA; Bivalacqua TJ; Manci EA; Diwan BA; Schimel DM; Cochard AE; Wang X; Schechter AN; Noguchi CT; Gladwin MT. 2007. Hemolysis in sickle cell mice causes pulmonary hypertension due to global impairment in nitric oxide bioavailability. Blood 109(7):3088-98. [PubMed: 17158223]  [MGI Ref ID J:145347]

Ieremia J; Blau CA. 2002. Limitations of a mouse model of sickle cell anemia. Blood Cells Mol Dis 28(2):146-51. [PubMed: 12064910]  [MGI Ref ID J:128113]

Jang JE; Hidalgo A; Frenette PS. 2012. Intravenous immunoglobulins modulate neutrophil activation and vascular injury through FcgammaRIII and SHP-1. Circ Res 110(8):1057-66. [PubMed: 22415018]  [MGI Ref ID J:212521]

Kaul DK; Fabry ME; Suzuka SM; Zhang X. 2013. Antisickling fetal hemoglobin reduces hypoxia-inducible factor-1alpha expression in normoxic sickle mice: microvascular implications. Am J Physiol Heart Circ Physiol 304(1):H42-50. [PubMed: 23125209]  [MGI Ref ID J:192836]

Kaul DK; Liu XD; Chang HY; Nagel RL; Fabry ME. 2004. Effect of fetal hemoglobin on microvascular regulation in sickle transgenic-knockout mice. J Clin Invest 114(8):1136-45. [PubMed: 15489961]  [MGI Ref ID J:93424]

Kiefmann R; Rifkind JM; Nagababu E; Bhattacharya J. 2008. Red blood cells induce hypoxic lung inflammation. Blood 111(10):5205-14. [PubMed: 18270324]  [MGI Ref ID J:135569]

Kohli DR; Li Y; Khasabov SG; Gupta P; Kehl LJ; Ericson ME; Nguyen J; Gupta V; Hebbel RP; Simone DA; Gupta K. 2010. Pain-related behaviors and neurochemical alterations in mice expressing sickle hemoglobin: modulation by cannabinoids. Blood 116(3):456-65. [PubMed: 20304807]  [MGI Ref ID J:162824]

Lagoda G; Sezen SF; Hurt KJ; Cabrini MR; Mohanty DK; Burnett AL. 2014. Sustained nitric oxide (NO)-releasing compound reverses dysregulated NO signal transduction in priapism. FASEB J 28(1):76-84. [PubMed: 24076963]  [MGI Ref ID J:206623]

Lebensburger JD; Howard T; Hu Y; Pestina TI; Gao G; Johnson M; Zakharenko SS; Ware RE; Tuomanen EI; Persons DA; Rosch JW. 2012. Hydroxyurea therapy of a murine model of sickle cell anemia inhibits the progression of pneumococcal disease by down-modulating E-selectin. Blood 119(8):1915-21. [PubMed: 22130804]  [MGI Ref ID J:181749]

Levasseur DN; Ryan TM; Pawlik KM; Townes TM. 2003. Correction of a mouse model of sickle cell disease: lentiviral/antisickling beta-globin gene transduction of unmobilized, purified hematopoietic stem cells. Blood 102(13):4312-9. [PubMed: 12933581]  [MGI Ref ID J:134982]

Li J; Kim K; Hahm E; Molokie R; Hay N; Gordeuk VR; Du X; Cho J. 2014. Neutrophil AKT2 regulates heterotypic cell-cell interactions during vascular inflammation. J Clin Invest 124(4):1483-96. [PubMed: 24642468]  [MGI Ref ID J:209590]

Manci EA; Hillery CA; Bodian CA; Zhang ZG; Lutty GA; Coller BS. 2006. Pathology of Berkeley sickle cell mice: similarities and differences with human sickle cell disease. Blood 107(4):1651-8. [PubMed: 16166585]  [MGI Ref ID J:129356]

Meiler SE; Wade M; Kutlar F; Yerigenahally SD; Xue Y; Moutouh-de Parseval LA; Corral LG; Swerdlow PS; Kutlar A. 2011. Pomalidomide augments fetal hemoglobin production without the myelosuppressive effects of hydroxyurea in transgenic sickle cell mice. Blood 118(4):1109-12. [PubMed: 21536862]  [MGI Ref ID J:174901]

Mi T; Abbasi S; Zhang H; Uray K; Chunn JL; Xia LW; Molina JG; Weisbrodt NW; Kellems RE; Blackburn MR; Xia Y. 2008. Excess adenosine in murine penile erectile tissues contributes to priapism via A2B adenosine receptor signaling. J Clin Invest 118(4):1491-501. [PubMed: 18340377]  [MGI Ref ID J:135978]

Nandedkar SD; Feroah TR; Hutchins W; Weihrauch D; Konduri KS; Wang J; Strunk RC; DeBaun MR; Hillery CA; Pritchard KA. 2008. Histopathology of experimentally induced asthma in a murine model of sickle cell disease. Blood 112(6):2529-38. [PubMed: 18579795]  [MGI Ref ID J:138890]

Noguchi CT; Gladwin M; Diwan B; Merciris P; Smith R; Yu X; Buzard G; Fitzhugh A; Keefer LK; Schechter AN; Mohandas N. 2001. Pathophysiology of a sickle cell trait mouse model: human alpha(beta)(S) transgenes with one mouse beta-globin allele. Blood Cells Mol Dis 27(6):971-7. [PubMed: 11831863]  [MGI Ref ID J:128124]

Prado GN; Romero JR; Rivera A. 2013. Endothelin-1 receptor antagonists regulate cell surface-associated protein disulfide isomerase in sickle cell disease. FASEB J 27(11):4619-29. [PubMed: 23913858]  [MGI Ref ID J:203687]

Pritchard KA Jr; Feroah TR; Nandedkar SD; Holzhauer SL; Hutchins W; Schulte ML; Strunk RC; Debaun MR; Hillery CA. 2012. Effects of experimental asthma on inflammation and lung mechanics in sickle cell mice. Am J Respir Cell Mol Biol 46(3):389-96. [PubMed: 22033263]  [MGI Ref ID J:194579]

Pritchard KA Jr; Ou J; Ou Z; Shi Y; Franciosi JP; Signorino P; Kaul S; Ackland-Berglund C; Witte K; Holzhauer S; Mohandas N; Guice KS; Oldham KT; Hillery CA. 2004. Hypoxia-induced acute lung injury in murine models of sickle cell disease. Am J Physiol Lung Cell Mol Physiol 286(4):L705-14. [PubMed: 12972407]  [MGI Ref ID J:134696]

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Sparkenbaugh EM; Chantrathammachart P; Mickelson J; van Ryn J; Hebbel RP; Monroe DM; Mackman N; Key NS; Pawlinski R. 2014. Differential contribution of FXa and thrombin to vascular inflammation in a mouse model of sickle cell disease. Blood 123(11):1747-56. [PubMed: 24449213]  [MGI Ref ID J:209655]

Sundaram N; Tailor A; Mendelsohn L; Wansapura J; Wang X; Higashimoto T; Pauciulo MW; Gottliebson W; Kalra VK; Nichols WC; Kato GJ; Malik P. 2010. High levels of placenta growth factor in sickle cell disease promote pulmonary hypertension. Blood 116(1):109-12. [PubMed: 20335221]  [MGI Ref ID J:162823]

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Health & husbandry

Health & Colony Maintenance Information

Animal Health Reports

Room Number           AX11

Colony Maintenance

Breeding & HusbandryThe breeding strategy utilized by The Jackson Laboratory follows a general scheme of mating non-sickling females with sickling males:

Type 1: homozygous for Hbatm1Paz, heterozygous for Hbbtm1Tow, carrying the transgene (-/-, +/-, Tg/?, non-sickling)
Type 2: homozygous for Hbatm1Paz, homozygous for Hbbtm1Tow, carrying the transgene (-/-, -/-, Tg/?, sickling)

Currently, trio matings are being used, with one sickling male rotating among 4 pens containing non-sickling females to maximize colony breeding. Sickling females are not suitable for breeding. Approximately 50% of progeny are homozygous for both Hba and Hbb targeted alleles and are hemizygous or homozygous for the transgene (sickling mutant mice). Of these sickling mutant mice, 81% will be hemizygous for the transgene and the other 19% will be homozygous. Only 40% of the sickling mutants will be male. Typically, ~20% of sickling mutant mice die between weaning and 14 weeks.

Breeding units supplied to the customer will consist of one non-sickling female (Type 1) and one sickling male (Type 2). Hemizygosity (Tg/0) or homozygosity (Tg/Tg) of the transgene will be designated when animals are shipped. Mating mice that are both homozygous for the transgene results in undersized litters and a small percentage of sickling pups.

Mating SystemSee Colony Maintenance under the Health & Care tab         (Female x Male)   01-MAR-06
Diet Information LabDiet® 5K52/5K67

Pricing and Purchasing

Pricing, Supply Level & Notes, Controls


Pricing for USA, Canada and Mexico shipping destinations View International Pricing

Live Mice

Price per mouse (US dollars $)GenderGenotypes Provided
Individual Mouse $320.00Female or MaleHomozygous for Hbatm1Paz, Heterozygous for Hbbtm1Tow, Hemizygous for Tg(HBA-HBBs)41Paz  
$320.00Female or MaleHomozygous for Hbatm1Paz, Heterozygous for Hbbtm1Tow, Homozygous for Tg(HBA-HBBs)41Paz  
Individual Mouse $320.00Female or MaleHomozygous for Hbatm1Paz, Homozygous for Hbbtm1Tow, Hemizygous for Tg(HBA-HBBs)41Paz  
Price per Pair (US dollars $)Pair Genotype
$640.00Homozygous for Hbatm1Paz, Heterozygous for Hbbtm1Tow, Hemizygous for Tg(HBA-HBBs)41Paz x Homozygous for Hbatm1Paz, Homozygous for Hbbtm1Tow, Hemizygous for Tg(HBA-HBBs)41Paz  

Standard Supply

Repository-Live.
Repository-Live represents an exclusive set of over 1800 unique mouse models across a vast array of research areas. Breeding colonies provide mice for large and small orders and fluctuate in size depending on current research demand. If a strain is not immediately available, you will receive an estimated availability timeframe for your inquiry or order in 2-3 business days. Repository strains typically are delivered at 4 to 8 weeks of age. Requests for specific ages will be noted but not guaranteed and we do not accept age requests for breeder pairs. However, if cohorts of mice (5 or more of one gender) are needed at a specific age range for experiments, we will do our best to accommodate your age request.

Pricing for International shipping destinations View USA Canada and Mexico Pricing

Live Mice

Price per mouse (US dollars $)GenderGenotypes Provided
Individual Mouse $416.00Female or MaleHomozygous for Hbatm1Paz, Heterozygous for Hbbtm1Tow, Hemizygous for Tg(HBA-HBBs)41Paz  
$416.00Female or MaleHomozygous for Hbatm1Paz, Heterozygous for Hbbtm1Tow, Homozygous for Tg(HBA-HBBs)41Paz  
Individual Mouse $416.00Female or MaleHomozygous for Hbatm1Paz, Homozygous for Hbbtm1Tow, Hemizygous for Tg(HBA-HBBs)41Paz  
Price per Pair (US dollars $)Pair Genotype
$832.00Homozygous for Hbatm1Paz, Heterozygous for Hbbtm1Tow, Hemizygous for Tg(HBA-HBBs)41Paz x Homozygous for Hbatm1Paz, Homozygous for Hbbtm1Tow, Hemizygous for Tg(HBA-HBBs)41Paz  

Standard Supply

Repository-Live.
Repository-Live represents an exclusive set of over 1800 unique mouse models across a vast array of research areas. Breeding colonies provide mice for large and small orders and fluctuate in size depending on current research demand. If a strain is not immediately available, you will receive an estimated availability timeframe for your inquiry or order in 2-3 business days. Repository strains typically are delivered at 4 to 8 weeks of age. Requests for specific ages will be noted but not guaranteed and we do not accept age requests for breeder pairs. However, if cohorts of mice (5 or more of one gender) are needed at a specific age range for experiments, we will do our best to accommodate your age request.

View USA Canada and Mexico Pricing View International Pricing

Standard Supply

Repository-Live.
Repository-Live represents an exclusive set of over 1800 unique mouse models across a vast array of research areas. Breeding colonies provide mice for large and small orders and fluctuate in size depending on current research demand. If a strain is not immediately available, you will receive an estimated availability timeframe for your inquiry or order in 2-3 business days. Repository strains typically are delivered at 4 to 8 weeks of age. Requests for specific ages will be noted but not guaranteed and we do not accept age requests for breeder pairs. However, if cohorts of mice (5 or more of one gender) are needed at a specific age range for experiments, we will do our best to accommodate your age request.

Control Information

  Control
   See control note: Homozygous for Hbatm1Paz, Heterozygous for Hbbtm1Tow, Hemizygous for Tg(HBA-HBBs)41Paz (non-sickling females from the colony)
   000664 C57BL/6J (approximate)
 
  Considerations for Choosing Controls
  Control Pricing Information for Genetically Engineered Mutant Strains.
 

Payment Terms and Conditions

Terms are granted by individual review and stated on the customer invoice(s) and account statement. These transactions are payable in U.S. currency within the granted terms. Payment for services, products, shipping containers, and shipping costs that are rendered are expected within the payment terms indicated on the invoice or stated by contract. Invoices and account balances in arrears of stated terms may result in The Jackson Laboratory pursuing collection activities including but not limited to outside agencies and court filings.


See Terms of Use tab for General Terms and Conditions


The Jackson Laboratory's Genotype Promise

The Jackson Laboratory has rigorous genetic quality control and mutant gene genotyping programs to ensure the genetic background of JAX® Mice strains as well as the genotypes of strains with identified molecular mutations. JAX® Mice strains are only made available to researchers after meeting our standards. However, the phenotype of each strain may not be fully characterized and/or captured in the strain data sheets. Therefore, we cannot guarantee a strain's phenotype will meet all expectations. To ensure that JAX® Mice will meet the needs of individual research projects or when requesting a strain that is new to your research, we suggest ordering and performing tests on a small number of mice to determine suitability for your particular project.
Ordering Information
JAX® Mice
Surgical and Preconditioning Services
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Tel: 1-800-422-6423 or 1-207-288-5845
Fax: 1-207-288-6150
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Terms of Use

Terms of Use


General Terms and Conditions


For Licensing and Use Restrictions view the link(s) below:
- Use of MICE by companies or for-profit entities requires a license prior to shipping.

Contact information

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Contracts Administration

phone:207-288-6470

JAX® Mice, Products & Services Conditions of Use

"MICE" means mouse strains, their progeny derived by inbreeding or crossbreeding, unmodified derivatives from mouse strains or their progeny supplied by The Jackson Laboratory ("JACKSON"). "PRODUCTS" means biological materials supplied by JACKSON, and their derivatives. "RECIPIENT" means each recipient of MICE, PRODUCTS, or services provided by JACKSON including each institution, its employees and other researchers under its control. MICE or PRODUCTS shall not be: (i) used for any purpose other than the internal research, (ii) sold or otherwise provided to any third party for any use, or (iii) provided to any agent or other third party to provide breeding or other services. Acceptance of MICE or PRODUCTS from JACKSON shall be deemed as agreement by RECIPIENT to these conditions, and departure from these conditions requires JACKSON's prior written authorization.

No Warranty

MICE, PRODUCTS AND SERVICES ARE PROVIDED “AS IS”. JACKSON EXTENDS NO WARRANTIES OF ANY KIND, EITHER EXPRESS, IMPLIED, OR STATUTORY, WITH RESPECT TO MICE, PRODUCTS OR SERVICES, INCLUDING ANY IMPLIED WARRANTY OF MERCHANTABILITY OR FITNESS FOR A PARTICULAR PURPOSE, OR ANY WARRANTY OF NON-INFRINGEMENT OF ANY PATENT, TRADEMARK, OR OTHER INTELLECTUAL PROPERTY RIGHTS.

In case of dissatisfaction for a valid reason and claimed in writing by a purchaser within ninety (90) days of receipt of mice, products or services, JACKSON will, at its option, provide credit or replacement for the mice or product received or the services provided.

No Liability

In no event shall JACKSON, its trustees, directors, officers, employees, and affiliates be liable for any causes of action or damages, including any direct, indirect, special, or consequential damages, arising out of the provision of MICE, PRODUCTS or services, including economic damage or injury to property and lost profits, and including any damage arising from acts or negligence on the part of JACKSON, its agents or employees. Unless prohibited by law, in purchasing or receiving MICE, PRODUCTS or services from JACKSON, purchaser or recipient, or any party claiming by or through them, expressly releases and discharges JACKSON from all such causes of action or damages, and further agrees to defend and indemnify JACKSON from any costs or damages arising out of any third party claims.

MICE and PRODUCTS are to be used in a safe manner and in accordance with all applicable governmental rules and regulations.

The foregoing represents the General Terms and Conditions applicable to JACKSON’s MICE, PRODUCTS or services. In addition, special terms and conditions of sale of certain MICE, PRODUCTS or services may be set forth separately in JACKSON web pages, catalogs, price lists, contracts, and/or other documents, and these special terms and conditions shall also govern the sale of these MICE, PRODUCTS and services by JACKSON, and by its licensees and distributors.

Acceptance of delivery of MICE, PRODUCTS or services shall be deemed agreement to these terms and conditions. No purchase order or other document transmitted by purchaser or recipient that may modify the terms and conditions hereof, shall be in any way binding on JACKSON, and instead the terms and conditions set forth herein, including any special terms and conditions set forth separately, shall govern the sale of MICE, PRODUCTS or services by JACKSON.


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