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- Description
- Phenotype
- Genes & alleles
- Genotyping
- Health & husbandry
- References
- Purchasing information
- Terms of Use
Description
Strain Information
Former Names B6C3F1/J-Tg(HD82Gln)81Dbo/J (Changed: 23-FEB-06 ) Type Mutant Stock; Transgenic; Additional information on Genetically Engineered and Mutant Mice. Visit our online Nomenclature tutorial. Mating System F1 x Hemizygote (Female x Male) 01-MAR-06 Species laboratory mouse Generation N29 (05-JAN-09) Donating Investigator David Borchelt, McKnight Brain Inst, Univ of Florida Appearance
agouti
Related Genotype: A/?
black
Related Genotype: a/aDescription
Mice expressing this transgene appear normal at birth through 1-2 months. Mice fail to gain weight, develop tremors, hypokinesis and lack coordination. They exhibit an abnormal gait and frequent hind limb clasping. Life expectancy is 5-6 months. Studies using huntingtin antibodies indicated numerous immunoreactive nuclear inclusions in multiple neuron populations. Neuritic damage is evident.Development
This transgenic line expresses an N-terminally truncated human huntingtin cDNA that encodes 82 glutamines and encompasses the first 171 amino acids. The altered huntingtin cDNA is under control of a mouse prion protein promoter. Expression is observed in neurons of the central nervous system.
Control Information
| Control | ||
|---|---|---|
| Noncarrier | ||
| Considerations for Choosing Controls | ||
Related Strains
Strains carrying other alleles of HTT
006471 B6.Cg-Tg(HDexon1)61Gpb/J 004360 B6;SJL-Tg(HD)63Aron/J 002809 B6CBA-Tg(HDexon1)61Gpb/1J 002810 B6CBA-Tg(HDexon1)62Gpb/1J 006494 B6CBA-Tg(HDexon1)62Gpb/3J 007578 CBy.Cg-Tg(HDexon1)61Gpb/J 004938 FVB-Tg(YAC128)53Hay/J 008197 FVB/N-Tg(HTT*97Q)IXwy/J 007247 FVB/N-Tg(YAC353G6)W7Hay/J 003640 FVB/NJ-Tg(YAC72)2511Hay/J View Strains carrying other alleles of HTT (10 strains)
Phenotype
Phenotype Information
assigned by genotype
Tg(HD82Gln)81Dbo/0
involves: C3H/HeJ * C57BL/6
- life span-post-weaning/aging
- premature death (MGI Ref ID J:53797)
- lifespan is approximately 5-6 months
- behavior/neurological phenotype
- abnormal gait (MGI Ref ID J:53797)
- bradykinesia (MGI Ref ID J:53797)
- impaired coordination (MGI Ref ID J:53797)
- tremors (MGI Ref ID J:53797)
- nervous system phenotype
- abnormal hypothalamus morphology (MGI Ref ID J:84682)
- neurodegeneration (MGI Ref ID J:84682)
- degenerated neurons in various hypothalamic areas including the lateral hypothalamus, the ventromedial hypothalamic nucleus, and the and the paraventricular nuclei
- neuronal intranuclear inclusions (MGI Ref ID J:53797)
- nuclear inclusions are observed in the cortex and caudate by immunocytochemistry
This mouse can be used to support research in many areas including:HTT related
Developmental Biology Research
Neurodevelopmental Defects
Mouse/Human Gene Homologs
Huntington's disease (chorea)
Neurobiology Research
Ataxia (Movement) Defects
Behavioral and Learning Defects
Cortical Defects
Huntington's disease
Neurodegeneration
Neurodevelopmental Defects
Neurotransmitter Receptor and Synaptic Vesicle Defects
Tremor Defects
Genes & Alleles
Gene & Allele Information
| Allele Symbol | Tg(HD82Gln)81Dbo | ||
|---|---|---|---|
| Allele Name | transgene insertion 81, David R Borchelt | ||
| Allele Type | Transgenic (random, expressed) | ||
| Common Name(s) | N171-82Q; TGN(HD82Gln)81Dbo; | ||
| Mutation Made By | David Borchelt, McKnight Brain Inst, Univ of Florida | ||
| Strain of Origin | C3H/B6 | ||
| Expressed Gene | HTT, huntingtin, human | ||
| Promoter | Prnp, prion protein, mouse, laboratory | ||
| Molecular Note | This transgenic line expresses an N-terminally truncated human huntingtin cDNA that encodes 82 glutamines and encompasses the first 171 amino acids. The altered huntingtin cDNA is under control of a mouse prion protein promoter, which drives transgene expression in the neurons of the central nervous system [MGI Ref ID J:53797] | ||
Genotyping
Genotyping Information
Genotyping Protocols
Tg(HD82Gln)81Dbo repeat assay, Standard PCR
Tg(HD82Gln)81Dbo, Standard PCR
Helpful Links
Genotyping resources and troubleshooting
References
References
Selected Reference(s)
Schilling G; Becher MW; Sharp AH; Jinnah HA; Duan K; Kotzuk JA; Slunt HH; Ratovitski T; Cooper JK; Jenkins NA; Copeland NG; Price DL; Ross CA; Borchelt DR. 1999. Intranuclear inclusions and neuritic aggregates in transgenic mice expressing a mutant N-terminal fragment of huntingtin [published erratum appears in Hum Mol Genet 1999 May;8(5):943] Hum Mol Genet 8(3):397-407. [PubMed: 9949199] [MGI Ref ID J:53797]
Additional References
Luthi-Carter R; Strand A; Peters NL; Solano SM; Hollingsworth ZR; Menon AS; Frey AS; Spektor BS; Penney EB; Schilling G; Ross CA; Borchelt DR; Tapscott SJ; Young AB; Cha JH; Olson JM. 2000. Decreased expression of striatal signaling genes in a mouse model of Huntington's disease. Hum Mol Genet 9(9):1259-71. [PubMed: 10814708] [MGI Ref ID J:62544]
Schilling G; Jinnah HA; Gonzales V; Coonfield ML; Kim Y; Wood JD; Price DL; Li XJ; Jenkins N; Copeland N; Moran T; Ross CA; Borchelt DR. 2001. Distinct behavioral and neuropathological abnormalities in transgenic mouse models of HD and DRPLA. Neurobiol Dis 8(3):405-18. [PubMed: 11442350] [MGI Ref ID J:70214]
Wheeler VC; Gutekunst CA; Vrbanac V; Lebel LA; Schilling G; Hersch S; Friedlander RM; Gusella JF; Vonsattel JP; Borchelt DR; MacDonald ME. 2002. Early phenotypes that presage late-onset neurodegenerative disease allow testing of modifiers in Hdh CAG knock-in mice. Hum Mol Genet 11(6):633-40. [PubMed: 11912178] [MGI Ref ID J:75831]
Yu ZX; Li SH; Evans J; Pillarisetti A; Li H; Li XJ. 2003. Mutant huntingtin causes context-dependent neurodegeneration in mice with Huntington's disease. J Neurosci 23(6):2193-202. [PubMed: 12657678] [MGI Ref ID J:82676]
Tg(HD82Gln)81Dbo relatedAndreassen OA; Dedeoglu A; Ferrante RJ; Jenkins BG; Ferrante KL; Thomas M; Friedlich A; Browne SE; Schilling G; Borchelt DR; Hersch SM; Ross CA; Beal MF. 2001. Creatine increase survival and delays motor symptoms in a transgenic animal model of Huntington's disease. Neurobiol Dis 8(3):479-91. [PubMed: 11447996] [MGI Ref ID J:70215]
Chaturvedi RK; Adhihetty P; Shukla S; Hennessy T; Calingasan N; Yang L; Starkov A; Kiaei M; Cannella M; Sassone J; Ciammola A; Squitieri F; Beal MF. 2009. Impaired PGC-1alpha function in muscle in Huntington's disease. Hum Mol Genet 18(16):3048-65. [PubMed: 19460884] [MGI Ref ID J:150738]
Ferrante RJ; Andreassen OA; Dedeoglu A; Ferrante KL; Jenkins BG; Hersch SM; Beal MF. 2002. Therapeutic effects of coenzyme Q10 and remacemide in transgenic mouse models of Huntington's disease. J Neurosci 22(5):1592-9. [PubMed: 11880489] [MGI Ref ID J:75156]
Jenkins BG; Andreassen OA; Dedeoglu A; Leavitt B; Hayden M; Borchelt D; Ross CA; Ferrante RJ; Beal MF. 2005. Effects of CAG repeat length, HTT protein length and protein context on cerebral metabolism measured using magnetic resonance spectroscopy in transgenic mouse models of Huntington's disease. J Neurochem 95(2):553-62. [PubMed: 16135087] [MGI Ref ID J:129784]
Li H; Wyman T; Yu ZX; Li SH; Li XJ. 2003. Abnormal association of mutant huntingtin with synaptic vesicles inhibits glutamate release. Hum Mol Genet 12(16):2021-30. [PubMed: 12913073] [MGI Ref ID J:85070]
Li SH; Yu ZX; Li CL; Nguyen HP; Zhou YX; Deng C; Li XJ. 2003. Lack of huntingtin-associated protein-1 causes neuronal death resembling hypothalamic degeneration in Huntington's disease. J Neurosci 23(17):6956-64. [PubMed: 12890790] [MGI Ref ID J:84682]
Luo S; Vacher C; Davies JE; Rubinsztein DC. 2005. Cdk5 phosphorylation of huntingtin reduces its cleavage by caspases: implications for mutant huntingtin toxicity. J Cell Biol 169(4):647-56. [PubMed: 15911879] [MGI Ref ID J:99654]
Luthi-Carter R; Strand A; Peters NL; Solano SM; Hollingsworth ZR; Menon AS; Frey AS; Spektor BS; Penney EB; Schilling G; Ross CA; Borchelt DR; Tapscott SJ; Young AB; Cha JH; Olson JM. 2000. Decreased expression of striatal signaling genes in a mouse model of Huntington's disease. Hum Mol Genet 9(9):1259-71. [PubMed: 10814708] [MGI Ref ID J:62544]
McBride JL; Ramaswamy S; Gasmi M; Bartus RT; Herzog CD; Brandon EP; Zhou L; Pitzer MR; Berry-Kravis EM; Kordower JH. 2006. Viral delivery of glial cell line-derived neurotrophic factor improves behavior and protects striatal neurons in a mouse model of Huntington's disease. Proc Natl Acad Sci U S A 103(24):9345-50. [PubMed: 16751280] [MGI Ref ID J:111040]
Miller VM; Nelson RF; Gouvion CM; Williams A; Rodriguez-Lebron E; Harper SQ; Davidson BL; Rebagliati MR; Paulson HL. 2005. CHIP suppresses polyglutamine aggregation and toxicity in vitro and in vivo. J Neurosci 25(40):9152-61. [PubMed: 16207874] [MGI Ref ID J:101343]
Olah J; Klivenyi P; Gardian G; Vecsei L; Orosz F; Kovacs GG; Westerhoff HV; Ovadi J. 2008. Increased glucose metabolism and ATP level in brain tissue of Huntington's disease transgenic mice. FEBS J 275(19):4740-55. [PubMed: 18721135] [MGI Ref ID J:142276]
Orr AL; Huang S; Roberts MA; Reed JC; Li S; Li XJ. 2008. Sex-dependent effect of BAG1 in ameliorating motor deficits of Huntington disease transgenic mice. J Biol Chem 283(23):16027-36. [PubMed: 18400759] [MGI Ref ID J:137552]
Qiu Z; Norflus F; Singh B; Swindell MK; Buzescu R; Bejarano M; Chopra R; Zucker B; Benn CL; DiRocco DP; Cha JH; Ferrante RJ; Hersch SM. 2006. Sp1 is up-regulated in cellular and transgenic models of Huntington disease, and its reduction is neuroprotective. J Biol Chem 281(24):16672-80. [PubMed: 16595660] [MGI Ref ID J:113725]
Ramaswamy S; McBride JL; Han I; Berry-Kravis EM; Zhou L; Herzog CD; Gasmi M; Bartus RT; Kordower JH. 2009. Intrastriatal CERE-120 (AAV-Neurturin) protects striatal and cortical neurons and delays motor deficits in a transgenic mouse model of Huntington's disease. Neurobiol Dis 34(1):40-50. [PubMed: 19150499] [MGI Ref ID J:147305]
Ravikumar B; Acevedo-Arozena A; Imarisio S; Berger Z; Vacher C; O'Kane CJ; Brown SD; Rubinsztein DC. 2005. Dynein mutations impair autophagic clearance of aggregate-prone proteins. Nat Genet 37(7):771-6. [PubMed: 15980862] [MGI Ref ID J:105219]
Schilling G; Jinnah HA; Gonzales V; Coonfield ML; Kim Y; Wood JD; Price DL; Li XJ; Jenkins N; Copeland N; Moran T; Ross CA; Borchelt DR. 2001. Distinct behavioral and neuropathological abnormalities in transgenic mouse models of HD and DRPLA. Neurobiol Dis 8(3):405-18. [PubMed: 11442350] [MGI Ref ID J:70214]
Tucci V; Achilli F; Blanco G; Lad HV; Wells S; Godinho S; Nolan PM. 2007. Reaching and grasping phenotypes in the mouse (Mus musculus): a characterization of inbred strains and mutant lines. Neuroscience 147(3):573-82. [PubMed: 17574766] [MGI Ref ID J:124220]
Wang CE; Tydlacka S; Orr AL; Yang SH; Graham RK; Hayden MR; Li S; Chan AW; Li XJ. 2008. Accumulation of N-terminal mutant huntingtin in mouse and monkey models implicated as a pathogenic mechanism in Huntington's disease. Hum Mol Genet 17(17):2738-51. [PubMed: 18558632] [MGI Ref ID J:138148]
Wang CE; Zhou H; McGuire JR; Cerullo V; Lee B; Li SH; Li XJ. 2008. Suppression of neuropil aggregates and neurological symptoms by an intracellular antibody implicates the cytoplasmic toxicity of mutant huntingtin. J Cell Biol 181(5):803-16. [PubMed: 18504298] [MGI Ref ID J:137025]
Wang J; Martin E; Gonzales V; Borchelt DR; Lee MK. 2008. Differential regulation of small heat shock proteins in transgenic mouse models of neurodegenerative diseases. Neurobiol Aging 29(4):586-97. [PubMed: 17316906] [MGI Ref ID J:135061]
Weydt P; Pineda VV; Torrence AE; Libby RT; Satterfield TF; Lazarowski ER; Gilbert ML; Morton GJ; Bammler TK; Strand AD; Cui L; Beyer RP; Easley CN; Smith AC; Krainc D; Luquet S; Sweet IR; Schwartz MW; La Spada AR. 2006. Thermoregulatory and metabolic defects in Huntington's disease transgenic mice implicate PGC-1alpha in Huntington's disease neurodegeneration. Cell Metab 4(5):349-62. [PubMed: 17055784] [MGI Ref ID J:129751]
Wheeler VC; Gutekunst CA; Vrbanac V; Lebel LA; Schilling G; Hersch S; Friedlander RM; Gusella JF; Vonsattel JP; Borchelt DR; MacDonald ME. 2002. Early phenotypes that presage late-onset neurodegenerative disease allow testing of modifiers in Hdh CAG knock-in mice. Hum Mol Genet 11(6):633-40. [PubMed: 11912178] [MGI Ref ID J:75831]
Yu ZX; Li SH; Evans J; Pillarisetti A; Li H; Li XJ. 2003. Mutant huntingtin causes context-dependent neurodegeneration in mice with Huntington's disease. J Neurosci 23(6):2193-202. [PubMed: 12657678] [MGI Ref ID J:82676]
Health & husbandry
Health & Colony Maintenance Information
Animal Health Reports
Room Number AX12
Colony Maintenance
Breeding & Husbandry This strain originated on a C3H/B6 background. The strain is maintained by crossing hemizygous males with wildtype B6C3F1/J females. The donating investigator reports that hemizygous females tend to make poor mothers. Expected coat color from breeding:Agouti and Black Mating System F1 x Hemizygote (Female x Male) 01-MAR-06 Diet Information LabDiet® 5K52/5K67
Purchasing information
Pricing, Supply Level & Notes, Controls, General Terms & Conditions
Pricing
| Pricing for USA, Canada and Mexico shipping destinations |
|
Weeks of Age Price (US dollars $) Gender Genotypes Provided Individual Mouse $239.00 Female or Male Hemizygous for Tg(HD82Gln)81Dbo
Pairs /Price (US dollars $) Pair Genotype $258.80 B6C3F1/J (100010) x Hemizygous for Tg(HD82Gln)81Dbo
Additional Supply Details
| Pricing for International shipping destinations |
|
Weeks of Age Price (US dollars $) Gender Genotypes Provided Individual Mouse $310.70 Female or Male Hemizygous for Tg(HD82Gln)81Dbo
Pairs /Price (US dollars $) Pair Genotype $336.50 B6C3F1/J (100010) x Hemizygous for Tg(HD82Gln)81Dbo
Additional Supply Details
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Supply Details
| Standard Supply | Repository-Live. A collection of over 1000 strains maintained as live colonies. Individual colonies are sized to meet current customer demand. Delivery for orders of 10 mice or less ranges on average from one to eight weeks; mice are generally shipped between four to six weeks of age with a maximum shipping age of approximately nine weeks. Colony sizes do not generally support stringent age specifications for large volumes of mice; however custom orders and larger quantities of mice are easily arranged. Estimated ship dates for all orders provided within two business days following order placement. |
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| Supply Notes |
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Control Information
| Control | ||
|---|---|---|
| Noncarrier | ||
| 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. | ||
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