Strain Name:

B6EiC3Sn a/A-Otcspf-ash/J

Stock Number:

001811

Availability:

Cryopreserved - Ready for recovery

Description

The genotypes of the animals provided may not reflect those discussed in the strain description or the mating scheme utilized by The Jackson Laboratory prior to cryopreservation. Please inquire for possible genotypes for this specific strain.

Strain Information

Type Mutant Stock; Spontaneous Mutation;
Additional information on Genetically Engineered and Mutant Mice.
Visit our online Nomenclature tutorial.
Specieslaboratory mouse
GenerationN46F1pN1

Appearance
black, small body size, sparse fur
Related Genotype: a/a Otcspf-ash/Y

agouti, small body size, sparse fur
Related Genotype: A/? Otcspf-ash/Y

black, normal fur and body size
Related Genotype: a/a +/? or a/a +/Y

agouti, normal fur and body size
Related Genotype: A/? +/? or A/? +/Y

Control Information

  Control
   Wild-type from the colony
 
  Considerations for Choosing Controls

Related Strains

View Strains carrying   A     (17 strains)

Strains carrying   a allele
003879   B10;TFLe-a/a T tf/+ tf/J
001538   B6 x B6C3Sn a/A-T(1;9)27H/J
000916   B6 x B6C3Sn a/A-T(5;12)31H/J
000602   B6 x B6C3Sn a/A-T(8;16)17H/J
000618   B6 x FSB/GnEi a/a Ctslfs/J
000577   B6 x STOCK a Oca2p Hps5ru2 Ednrbs/J
000601   B6 x STOCK a/a T(7;18)50H/J
000592   B6 x STOCK T(2;4)13H a/J
000001   B6.C3 A/a Mgrn1md/J
000231   B6;C3Fe a/a-Csf1op/J
000785   B6;D2-a Es1e/EiJ
000604   B6C3 a/A-T(10;13)199H +/+ Lystbg-J/J or Lystbg-2J/J
002807   B6C3Fe a/a-Meox2fla/J
000224   B6C3Fe a/a-Scyl1mdf/J
001037   B6C3Fe a/a-Agtpbp1pcd/J
000221   B6C3Fe a/a-Alx4lst-J/J
002062   B6C3Fe a/a-Atp7aMo-8J/J
001756   B6C3Fe a/a-Cacng2stg/J
001815   B6C3Fe a/a-Col1a2oim/J
000209   B6C3Fe a/a-Dh/J
000211   B6C3Fe a/a-Dstdt-J/J
000210   B6C3Fe a/a-Edardl-J/J
000207   B6C3Fe a/a-Edaraddcr/J
000182   B6C3Fe a/a-Eef1a2wst/J
001278   B6C3Fe a/a-Glra1spd/J
000241   B6C3Fe a/a-Glrbspa/J
002875   B6C3Fe a/a-Hoxd13spdh/J
000304   B6C3Fe a/a-Krt71Ca Scn8amed-J/J
000226   B6C3Fe a/a-Largemyd/J
000636   B6C3Fe a/a-Lmx1adr-J/J
001280   B6C3Fe a/a-Lse/J
001573   B6C3Fe a/a-MitfMi/J
001035   B6C3Fe a/a-Napahyh/J
000181   B6C3Fe a/a-Otogtwt/J
000278   B6C3Fe a/a-Papss2bm Hps1ep Hps6ru/J
000205   B6C3Fe a/a-Papss2bm/J
002078   B6C3Fe a/a-Pcdh15av-2J/J
000246   B6C3Fe a/a-Pitpnavb/J
001430   B6C3Fe a/a-Ptch1mes/J
000506   B6C3Fe a/a-Qkqk/J
000235   B6C3Fe a/a-Relnrl/J
000237   B6C3Fe a/a-Rorasg/J
000290   B6C3Fe a/a-Sox10Dom/J
000230   B6C3Fe a/a-Tcirg1oc/J
003612   B6C3Fe a/a-Trak1hyrt/J
001512   B6C3Fe a/a-Ttnmdm/J
001607   B6C3Fe a/a-Unc5crcm/J
000005   B6C3Fe a/a-Wc/J
000243   B6C3Fe a/a-Wnt1sw/J
000248   B6C3Fe a/a-Xpl/J
001750   B6C3Fe a/a-XsJ/J
000624   B6C3Fe a/a-anx/J
008044   B6C3Fe a/a-bpck/J
003020   B6C3Fe a/a-dep/J
002018   B6C3Fe a/a-din/J
002339   B6C3Fe a/a-nma/J
000240   B6C3Fe a/a-soc/J
000063   B6C3Fe a/a-sy/J
001055   B6C3Fe a/a-tip/J
000245   B6C3Fe a/a-tn/J
000296   B6C3Fe-a/a Hoxa13Hd Mcoln3Va-J/J
000019   B6C3Fe-a/a-Itpr1opt/J
001022   B6C3FeF1/J a/a
006450   B6EiC3 a/A-Vss/GrsrJ
000971   B6EiC3 a/A-Och/J
000551   B6EiC3 a/A-Tbx15de-H/J
000557   B6EiC3-+ a/LnpUl A/J
000503   B6EiC3Sn a/A-Gy/J
002343   B6EiC3Sn a/A-Otcspf/J
000391   B6EiC3Sn a/A-Pax6Sey-Dey/J
001924   B6EiC3Sn a/A-Ts(1716)65Dn
001923   B6EiC3Sn a/A-Ts(417)2Lws TimT(4;17)3Lws/J
000225   C3FeLe.B6 a/a-Ptpn6me/J
008425   C3FeLe.B6-a Trl/J
000198   C3FeLe.B6-a/J
000291   C3FeLe.Cg-a/a Hm KitlSl Krt71Ca-J/J
001886   C3HeB/FeJLe a/a-gnd/J
000584   C57BL/6J-+ T(1;2)5Ca/a +/J
000284   CWD/LeJ
000670   DBA/1J
000671   DBA/2J
001057   HPT/LeJ
000260   JGBF/LeJ
000265   MY/HuLeJ
000308   SSL/LeJ
000994   STOCK a Myo5ad Mregdsu/J
000064   STOCK a Tyrp1b Sisi/J
002238   STOCK a Tyrp1b shmy/J
001433   STOCK a skt/J
000579   STOCK a tp/J
000319   STOCK a us/J
002648   STOCK a/a Cln6nclf/J
000317   STOCK a/a Egfrwa2/J
000302   STOCK a/a MitfMi-wh +/+ Itpr1opt/J
000286   STOCK a/a Myo5ad fd/+ +/J
000206   STOCK a/a Tyrc-h/J
001432   STOCK a/a Tyrp1b sks/Tyrp1b +/J
000281   STOCK a/a ma Flgft/ma Flgft/J
000312   STOCK stb + a/+ Fignfi a/J
000596   STOCK T(2;11)30H/+ x AEJ-a Gdf5bp-H/J or A/J-a Gdf5bp-J/J
000970   STOCK T(2;16)28H A/T(2;16)28H a/J
000590   STOCK T(2;4)1Sn a/J
000594   STOCK T(2;8)26H a/T(2;8)26H a Tyrp1+/Tyrp1b/J
000623   TR/DiEiJ
View Strains carrying   a     (104 strains)

Strains carrying other alleles of Otc
000507   B6 x B6EiC3 a/A-Otcspf/J
002343   B6EiC3Sn a/A-Otcspf/J
001672   C57BL/6J-Otcspf-J/J
View Strains carrying other alleles of Otc     (3 strains)

Strains carrying other alleles of a
000251   AEJ.Cg-ae +/a Gdf5bp-H/J
000202   AEJ/Gn-bd/J
000199   AEJ/GnLeJ
000433   B10.C-H3c H13? A/(28NX)SnJ
000427   B10.CE-H13b Aw/(30NX)SnJ
000423   B10.KR-H13? A/SnJ
000420   B10.LP-H13b Aw/Sn
000477   B10.PA-Pldnpa H3e at/SnJ
000419   B10.UW-H3b we Pax1un at/SnJ
000593   B6 x B6CBCa Aw-J/A-Grid2Lc T(2;6)7Ca MitfMi-wh/J
000502   B6 x B6CBCa Aw-J/A-Myo5aflr Gnb5flr/J
000599   B6 x B6CBCa Aw-J/A-T(5;13)264Ca KitW-v/J
003759   B6 x B6EiC3Sn a/A-T(10;16)232Dn/J
002071   B6 x B6EiC3Sn a/A-T(11;17)202Dn/J
002113   B6 x B6EiC3Sn a/A-T(11A2;16B3)238Dn/J
002068   B6 x B6EiC3Sn a/A-T(11B1;16B5)233Dn/J
002069   B6 x B6EiC3Sn a/A-T(14E4or5;16B5)225Dn/J
001926   B6 x B6EiC3Sn a/A-T(15;16)198Dn/J
001832   B6 x B6EiC3Sn a/A-T(15E;16B1)60Dn/J
003758   B6 x B6EiC3Sn a/A-T(16C3-4;17A2)65Dn/J
001833   B6 x B6EiC3Sn a/A-T(1C2;16C3)45Dn/J
001903   B6 x B6EiC3Sn a/A-T(6F;18C)57Dn/J
001535   B6 x B6EiC3Sn a/A-T(8A4;12D1)69Dn/J
001831   B6 x B6EiC3Sn a/A-T(8C3;16B5)164Dn/J
002016   B6(Cg)-Aw-J EdaTa-6J Chr YB6-Sxr/EiJ
000552   B6-Aw-J-EdaTa-6J.Cg-Sxr
001730   B6-Aw-J-EdaTa-6J.Cg-Sxrb Hya-/J
000841   B6-Aw-J.CBy-EdaTa-By/J
001809   B6-Aw-J.Cg-EdaTa-6J +/+ ArTfm/J
000600   B6-Gpi1b x B6CBCa Aw-J/A-T(7;15)9H Gpi1a/J
000769   B6.C/(HZ18)By-at-44J/J
000203   B6.C3-Aiy/a/J
000017   B6.C3-Avy/J
001572   B6.C3-am-J/J
000021   B6.Cg-Ay/J
100409   B6129PF1/J-Aw-J/Aw
004200   B6;CBACa Aw-J/A-Npr2cn-2J/GrsrJ
000505   B6C3 Aw-J/A-Mutedmu/J
000065   B6C3Fe a/a-we Pax1un at/J
000314   B6CBACa Aw-J/A-EdaTa/J-XO
000501   B6CBACa Aw-J/A-Aifm1Hq/J
001046   B6CBACa Aw-J/A-Grid2Lc/J
000500   B6CBACa Aw-J/A-Gs/J
002703   B6CBACa Aw-J/A-Hydinhy3/J
000247   B6CBACa Aw-J/A-Kcnj6wv/J
000287   B6CBACa Aw-J/A-Plp1jp EdaTa/J
000515   B6CBACa Aw-J/A-SfnEr/J
000242   B6CBACa Aw-J/A-spc/J
000288   B6CBACa Aw-J/A-we a Mafbkr/J
001201   B6CBACaF1/J-Aw-J/A
001752   B6CBCa Aw-J/A-T(7;15)9H/J
001875   B6EiC3SnF1/J
000200   C3FeB6 A/Aw-J-Ankank/J
000638   C3FeB6 A/Aw-J-Spnb4qv-J/J
001203   C3FeB6F1/J A/Aw-J
001272   C3H/HeSnJ-Ahvy/J
000099   C3HeB/FeJ-Avy/J
000338   C57BL/6J Aw-J-EdaTa-6J/J
000258   C57BL/6J-Ai/a/J
000774   C57BL/6J-Asy/a/J
000569   C57BL/6J-Aw-J-EdaTa +/+ ArTfm/J
000051   C57BL/6J-Aw-J/J
000055   C57BL/6J-at-33J/J
000070   C57BL/6J-atd/J
002468   KK.Cg-Ay/J
000262   LS/LeJ
001427   STOCK Aw us/J
View Strains carrying other alleles of a     (67 strains)

Phenotype

Phenotype Information

View Related Disease (OMIM) Terms

Related Disease (OMIM) Terms
Ornithine Transcarbamylase Deficiency, Hyperammonemia Due to - Models with phenotypic similarity to human disease where etiologies involve orthologs.1
1 Human genes are associated with this disease. Orthologs of those genes appear in the mouse genotype(s).
View Mammalian Phenotype Terms

Mammalian Phenotype Terms
      assigned by genotype

The following phenotype information may relate to a genetic background differing from this JAX® Mice strain.

Otcspf-ash/Otc+

        Background Not Specified
  • skin/coat/nails phenotype
  • partial hair loss (MGI Ref ID J:5476)
    • most heterozygous females appear normal but many show some hair loss

Otcspf-ash/Otc+

        involves: C57BL/6
  • homeostasis/metabolism phenotype
  • abnormal urine homeostasis (MGI Ref ID J:23205)
    • females excrete significantly higher concentrations of orotic acid than controls during starvation
    • under a nitrogen load of 5.6 g tryptone/kg body mass, urinary orotic acid excretion increases even more
  • renal/urinary system phenotype
  • abnormal urine homeostasis (MGI Ref ID J:23205)
    • females excrete significantly higher concentrations of orotic acid than controls during starvation
    • under a nitrogen load of 5.6 g tryptone/kg body mass, urinary orotic acid excretion increases even more

Otcspf-ash/Y

        Background Not Specified
  • skin/coat/nails phenotype
  • sparse hair (MGI Ref ID J:26977)
  • wrinkled skin (MGI Ref ID J:26977)
  • homeostasis/metabolism phenotype
  • abnormal enzyme/coenzyme activity (MGI Ref ID J:7199)
    • liver extracts from hemizygous males have 5-10% of normal ornithine transcarbamylase activity
    • mitochondrial specific activity of carbamoyl-phosphate synthetase (ammonia) and glutamate dehydrogenase is about 15% lower than in controls
    • citrate synthase activity is increased in the liver but specific activity in mitochondria is similar to controls
    • activities of beta-hydroxybutyrate dehydrogenase and cytochrome oxidase are 22% higher and 30% lower, respectively, in the liver
  • abnormal protein level (MGI Ref ID J:26976)
    • mutants show a 12% and 33% increase in total liver and mitochondrial protein content, respectively, compared to controls
  • abnormal urine homeostasis (MGI Ref ID J:26977)
    • orotic aciduria
  • increased circulating ammonia level (MGI Ref ID J:26977)
  • renal/urinary system phenotype
  • abnormal urine homeostasis (MGI Ref ID J:26977)
    • orotic aciduria
  • cellular phenotype
  • abnormal mitochondrial physiology (MGI Ref ID J:26976)
    • coupled and uncoupled mitochodria synthesize citrulline at unexpectedly high rates in the presence of 10 mM ornithine and the substrates for carbamoyl phosphate synthesis, however these rates are somewhat lower than rates of normal mitochondria; this difference arises from a lower carbamoyl-phosphate synthetase activity
    • at external ornithine concentrations of less than 2 mM, a smaller fraction of the carbamoyl phophate synthesized is converted into citrulline than in controls
  • liver/biliary system phenotype
  • enlarged liver (MGI Ref ID J:26976)
    • increased liver weight (MGI Ref ID J:26976)
      • liver weight per unit body weight is about 20% greater than normal

Otcspf-ash/Y

        involves: C57BL/6
  • homeostasis/metabolism phenotype
  • abnormal homeostasis (MGI Ref ID J:23205)
    • concentration of carbamylphosphate in the small intestine during starvation is about 3 times that of controls
    • abnormal urine homeostasis (MGI Ref ID J:23205)
      • males excrete significantly higher concentrations of orotic acid than controls during starvation
      • under a stressful condition of a nitrogen load of 5.6 g tryptone/kg body mass, urinary orotic acid excretion increases even more
  • immune system phenotype
  • increased susceptibility to parasitic infection (MGI Ref ID J:23205)
    • most mutants parasitized with mouse ticks (Myocoptes musculinus) do not survive
  • life span-post-weaning/aging
  • increased sensitivity to induced morbidity/mortality (MGI Ref ID J:23205)
    • most mutants parasitized with mouse ticks (Myocoptes musculinus) do not survive
  • renal/urinary system phenotype
  • abnormal urine homeostasis (MGI Ref ID J:23205)
    • males excrete significantly higher concentrations of orotic acid than controls during starvation
    • under a stressful condition of a nitrogen load of 5.6 g tryptone/kg body mass, urinary orotic acid excretion increases even more

Otcspf-ash/Y

        B6EiC3Sn a/A-Otcspf-ash/J
  • behavior/neurological phenotype
  • abnormal spatial learning (MGI Ref ID J:108979)
    • mutants are unable to locate the hidden platform by day 4 in the Morris water maze compared to wild-type which find it by this time
  • abnormal spatial reference memory (MGI Ref ID J:108979)
    • mutants are unable to locate the hidden platform by day 4 in the Morris water maze compared to wild-type which find it by this time
  • abnormal spatial working memory (MGI Ref ID J:108979)
    • in a two trial Y-maze, mutants show reduced retention memory, unable to distinguish the novel arm from the other two arms
  • growth/size phenotype
  • decreased body size (MGI Ref ID J:108979)
    • smaller than mature 6-7 month old wild-type mice
  • homeostasis/metabolism phenotype
  • increased circulating ammonia level (MGI Ref ID J:108979)
  • immune system phenotype
  • increased susceptibility to endotoxin shock (MGI Ref ID J:108979)
    • mutants are more sensitive to immune challenge with LPS than wild-type, with significant delay in recovery time from sickness
View Research Applications

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

Otcspf-ash related

Dermatology Research
Skin and Hair Texture Defects

Metabolism Research

Mouse/Human Gene Homologs
ornithine transcarbamylase deficiency

Genes & Alleles

Gene & Allele Information

 
Allele Symbol A
Allele Name wild type agouti
Allele Type Spontaneous
Common Name(s) dark-bellied agouti;
Strain of Originvarious
Gene Symbol and Name a, nonagouti
Chromosome 2
Gene Common Name(s) AGSW; AGTI; AGTIL; ASP; As; MGC126092; MGC126093; SHEP9; agouti; agouti signal protein; agouti suppressor;
General Note The A allele is usually regarded as a wild-type allele. Hairs are black with a subapical yellow band. This black-yellow-black pattern is referred to as agouti. The general appearance is yellowish brown, slightly lighter on the belly than on the back.
Molecular Note This allele, often referred to as wild-type, comprises a novel 131 amino acid protein encoded in a gene comprising four exons, three coding, spanning 18kb. Unique changes in this gene account for all other alleles that have been molecularly characterized. The expression of this allele is almost always dominant to other alleles of this gene. [MGI Ref ID J:3523]
 
Allele Symbol Otcspf-ash
Allele Name abnormal skin and hair
Allele Type Spontaneous
Common Name(s) spf-ash; spfash;
Gene Symbol and Name Otc, ornithine transcarbamylase
Chromosome X
Gene Common Name(s) AI265390; MGC108742; MGC129967; MGC129968; MGC138856; OCTD; Sf; Sparse-fur; expressed sequence AI265390; sparse fur; spf;
Molecular Note A G to A missense transition of the last nucleotide of exon 4 changes an arginine to histidine (R129H) and results in inefficient mRNA splicing at this site, as well as at a site located 48 bases into the adjacent intron. This allele is hypomorphic; approximately 5-10% of wild-type hepatic enzyme activity is retained. [MGI Ref ID J:9817]
 
Allele Symbol a
Allele Name nonagouti
Allele Type Spontaneous

Genotyping

Genotyping Information

This strain will not have a genotyping protocol or one is not currently available.

Helpful Links

Genotyping resources and troubleshooting

References

References

Additional References

de Jonge WJ; Hallemeesch MM; Kwikkers KL; Ruijter JM; de Gier-de Vries C; van Roon MA; Meijer AJ; Marescau B; de Deyn PP; Deutz NE; Lamers WH. 2002. Overexpression of arginase I in enterocytes of transgenic mice elicits a selective arginine deficiency and affects skin, muscle, and lymphoid development. Am J Clin Nutr 76(1):128-40. [PubMed: 12081826]  [MGI Ref ID J:80556]

A related

Blewitt ME; Vickaryous NK; Hemley SJ; Ashe A; Bruxner TJ; Preis JI; Arkell R; Whitelaw E. 2005. An N-ethyl-N-nitrosourea screen for genes involved in variegation in the mouse. Proc Natl Acad Sci U S A 102(21):7629-34. [PubMed: 15890782]  [MGI Ref ID J:99816]

Bultman SJ; Michaud EJ; Woychik RP. 1992. Molecular characterization of the mouse agouti locus. Cell 71(7):1195-204. [PubMed: 1473152]  [MGI Ref ID J:3523]

Bundschuh VG; Madry M. 1988. [atwp mutation in an albino mouse substrain (AB/Hum-1)] Z Versuchstierkd 31(6):249-54. [PubMed: 3227730]  [MGI Ref ID J:16568]

Czyzyk TA; Sikorski MA; Yang L; McKnight GS. 2008. Disruption of the RIIbeta subunit of PKA reverses the obesity syndrome of Agouti lethal yellow mice. Proc Natl Acad Sci U S A 105(1):276-81. [PubMed: 18172198]  [MGI Ref ID J:131039]

Dickie MM. 1969. Mutations at the agouti locus in the mouse. J Hered 60(1):20-5. [PubMed: 5798139]  [MGI Ref ID J:30922]

Dry FW. 1928. The agouti coloration of the mouse (Mus Musculus) and the rat (Mus Norvegicus). J Genet 20:131-144.  [MGI Ref ID J:46318]

Guido V, and The Mouse Mutant Resource (MMR) at The Jackson Laboratory. 2002. Two new mutations of white bellied agouti, w-46J and w-47J MGI Direct Data Submission :.  [MGI Ref ID J:77218]

Jackson IJ; Budd PS; Keighren M; McKie L. 2007. Humanized MC1R transgenic mice reveal human specific receptor function. Hum Mol Genet 16(19):2341-8. [PubMed: 17652101]  [MGI Ref ID J:129904]

Mather K; North SB. 1940. Umbrous: a case of dominance modification in mice. J Genet 40:229-41.  [MGI Ref ID J:280]

Perry WL; Copeland NG; Jenkins NA. 1994. The molecular basis for dominant yellow agouti coat color mutations. Bioessays 16(10):705-7. [PubMed: 7980472]  [MGI Ref ID J:21244]

Quevedo WC Jr.; Chase HB. 1958. An analysis of the light mutation of coat color in mice. J Morphol 102:329-345.  [MGI Ref ID J:13094]

Silvers WK. 1979. The Coat Colors of Mice; A Model for Mammalian Gene Action and Interaction. In: The Coat Colors of Mice. Springer-Verlag, New York.  [MGI Ref ID J:78801]

Otcspf-ash related

Bell P; Moscioni AD; McCarter RJ; Wu D; Gao G; Hoang A; Sanmiguel JC; Sun X; Wivel NA; Raper SE; Furth EE; Batshaw ML; Wilson JM. 2006. Analysis of tumors arising in male B6C3F1 mice with and without AAV vector delivery to liver. Mol Ther 14(1):34-44. [PubMed: 16682254]  [MGI Ref ID J:115067]

Cavard C; Grimber G; Dubois N; Chasse JF; Bennoun M; Minet-Thuriaux M; Kamoun P; Briand P. 1988. Correction of mouse ornithine transcarbamylase deficiency by gene transfer into the germ line. Nucleic Acids Res 16(5):2099-110. [PubMed: 3162766]  [MGI Ref ID J:26977]

Cohen NS; Cheung CW; Raijman L. 1989. Altered enzyme activities and citrulline synthesis in liver mitochondria from ornithine carbamoyltransferase-deficient sparse-furash mice. Biochem J 257(1):251-7. [PubMed: 2920015]  [MGI Ref ID J:26976]

Doolittle DP; Hulbert LL; Cordy C. 1974. A new allele of the sparse fur gene in the mouse. J Hered 65(3):194-5. [PubMed: 4603259]  [MGI Ref ID J:5476]

Hodges PE; Rosenberg LE. 1989. The spfash mouse: a missense mutation in the ornithine transcarbamylase gene also causes aberrant mRNA splicing. Proc Natl Acad Sci U S A 86(11):4142-6. [PubMed: 2471197]  [MGI Ref ID J:9817]

Li MX; Nakajima T; Fukushige T; Kobayashi K; Seiler N; Saheki T. 1999. Aberrations of ammonia metabolism in ornithine carbamoyltransferase-deficient spf-ash mice and their prevention by treatment with urea cycle intermediate amino acids and an ornithine aminotransferase inactivator. Biochim Biophys Acta 1455(1):1-11. [PubMed: 10524224]  [MGI Ref ID J:57489]

Luiking YC; Hallemeesch MM; van de Poll MC; Dejong CH; de Jonge WJ; Lamers WH; Deutz NE. 2008. Reduced citrulline availability by OTC deficiency in mice is related to reduced nitric oxide production. Am J Physiol Endocrinol Metab 295(6):E1315-22. [PubMed: 18697914]  [MGI Ref ID J:143948]

Marini JC; Broussard SR. 2006. Hyperammonemia increases sensitivity to LPS. Mol Genet Metab 88(2):131-7. [PubMed: 16497529]  [MGI Ref ID J:108979]

Marini JC; Erez A; Castillo L; Lee B. 2007. Interaction between murine spf-ash mutation and genetic background yields different metabolic phenotypes. Am J Physiol Endocrinol Metab 293(6):E1764-71. [PubMed: 17925451]  [MGI Ref ID J:130094]

Marini JC; Lee B; Garlick PJ. 2006. Ornithine restores ureagenesis capacity and mitigates hyperammonemia in Otc(spf-ash) mice. J Nutr 136(7):1834-8. [PubMed: 16772445]  [MGI Ref ID J:114947]

Marini JC; Lee B; Garlick PJ. 2006. Reduced ornithine transcarbamylase activity does not impair ureagenesis in Otc(spf-ash) mice. J Nutr 136(4):1017-20. [PubMed: 16549467]  [MGI Ref ID J:112832]

Rosenberg LE; Kalousek F; Orsulak MD. 1983. Biogenesis of ornithine transcarbamylase in spfash mutant mice: two cytoplasmic precursors, one mitochondrial enzyme. Science 222(4622):426-8. [PubMed: 6623083]  [MGI Ref ID J:7199]

Saheki T; Mori K; Kobayashi K; Horiuchi M; Shige T; Obara T; Suzuki S; Mori M; Yamamura K. 1995. Importance of ornithine transcarbamylase (OTC) deficiency in small intestine for urinary orotic acid excretion: analysis of OTC-deficient spf-ash mice with OTC transgene. Biochim Biophys Acta 1270(1):87-93. [PubMed: 7827141]  [MGI Ref ID J:23205]

Shiojiri N; Imai H; Goto S; Ohta T; Ogawa K; Mori M. 1997. Mosaic pattern of ornithine transcarbamylase expression in spfash mouse liver. Am J Pathol 151(2):413-21. [PubMed: 9250154]  [MGI Ref ID J:42523]

Shiojiri N; Inujima S; Ishikawa K; Terada K; Mori M. 2001. Cell lineage analysis during liver development using the spf(ash)-heterozygous mouse. Lab Invest 81(1):17-25. [PubMed: 11204270]  [MGI Ref ID J:67189]

Shiojiri N; Sano M; Inujima S; Nitou M; Kanazawa M; Mori M. 2000. Quantitative analysis of cell allocation during liver development, using the spf(ash)-heterozygous female mouse. Am J Pathol 156(1):65-75. [PubMed: 10623655]  [MGI Ref ID J:59364]

Sundberg JP (ed.). 1994. . In: Handbook of Mouse Mutations with Skin and Hair Abnormalities: Animal Models and Biomedical Tools. CRC Press, Boca Raton.  [MGI Ref ID J:30359]

Zimmer KP; Bendiks M; Mori M; Kominami E; Robinson MB; Ye X ; Wilson JM. 1999. Efficient mitochondrial import of newly synthesized ornithine transcarbamylase (OTC) and correction of secondary metabolic alterations in spf(ash) mice following gene therapy of OTC deficiency. Mol Med 5(4):244-53. [PubMed: 10448647]  [MGI Ref ID J:55968]

de Jonge WJ; Hallemeesch MM; Kwikkers KL; Ruijter JM; de Gier-de Vries C; van Roon MA; Meijer AJ; Marescau B; de Deyn PP; Deutz NE; Lamers WH. 2002. Overexpression of arginase I in enterocytes of transgenic mice elicits a selective arginine deficiency and affects skin, muscle, and lymphoid development. Am J Clin Nutr 76(1):128-40. [PubMed: 12081826]  [MGI Ref ID J:80556]

de Jonge WJ; Kwikkers KL; te Velde AA; van Deventer SJ; Nolte MA; Mebius RE; Ruijter JM; Lamers MC; Lamers WH. 2002. Arginine deficiency affects early B cell maturation and lymphoid organ development in transgenic mice. J Clin Invest 110(10):1539-48. [PubMed: 12438451]  [MGI Ref ID J:80204]

Health & husbandry

Health & Colony Maintenance Information

Currently there no information available for this strain. This may be due to the supply level of this strain.

Purchasing information

Pricing, Supply Level & Notes, Controls, General Terms & Conditions

Pricing

Pricing for USA, Canada and Mexico shipping destinations View International pricing
Price (US dollars $)
Cryorecovery Fee $1900.00
Animals Provided

At least two mice that carry the mutation (if it is a mutant strain) will be provided. Their genotypes may not reflect those discussed in the strain description. Please inquire for possible genotypes and see additional details below.

Additional Supply Details

Pricing for International shipping destinations View USA Canada and Mexico pricing
Price (US dollars $)
Cryorecovery Fee $2470.00
Animals Provided

At least two mice that carry the mutation (if it is a mutant strain) will be provided. Their genotypes may not reflect those discussed in the strain description. Please inquire for possible genotypes and see additional details below.

Additional Supply Details

Supply Details

Standard SupplyCryopreserved. Ready for recovery. Please refer to pricing and supply notes for further information.
Supply Notes
  • Cryorecovery - Standard.
    We will fulfill your order by providing at least two pair of mice, at least one animal of each pair carrying the mutation of interest. The total number of animals provided, their gender and genotype will vary. Please inquire if larger numbers of animals with specific genotype and genders are needed. Animals typically ship between 13 and 16 weeks from the date of your order. If a second cryorecovery is needed in order to provide the minimum number of animals, animals will ship within 25 weeks. IMPORTANT NOTE: The genotypes of animals provided may not reflect the mating scheme utilized by The Jackson Laboratory prior to cryopreservation, or that discussed in the strain description. Please inquire about possible genotypes which will be recovered for this specific strain. The Jackson Laboratory cannot guarantee the reproductive success of mice shipped to your facility. If the mice are lost after the first three days (post-arrival) or do not produce progeny at your facility, a new order and fee will be necessary.

    Cryorecovery to establish a Dedicated Supply for greater quantities of mice.
    Mice recovered can be used to establish a dedicated colony to contractually supply you mice according to your requirements. Price by quotation. For more information on Dedicated Supply, please contact JAX® Services, Tel: 1-800-422-6423 (from U.S.A., Canada or Puerto Rico only) or 1-207-288-5845 (from any location).

  • This strain is included in the Mouse Mutant Resource collection.
  • Genomic DNA is available for this strain from the Mouse DNA Resource.

Control Information

  Control
   Wild-type from the colony
 
  Considerations for Choosing Controls
  USA, Canada and Mexico - Control Pricing Information for Genetically Engineered Mutant Strains.
  International - 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.
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Contact Information
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Terms of Use

Terms of Use


General Terms and Conditions


Contact information

General inquiries

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phone:207-288-6470
fax:207-288-6655

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. 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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