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Transgene Detail
Symbol: Tg(HDexon1)61Gpb
Name: transgene insertion 61, Gillian Bates
MGI ID: MGI:2389466
Synonyms: HD R6/1, httm, R6/1
Transgene: Tg(HDexon1)61Gpb  Location: unknown  
Alliance: Tg(HDexon1)61Gpb page
Strain of Origin:  CBA x C57BL/6
Transgene Type:    Transgenic (Humanized sequence, Inserted expressed sequence)
Mutation:    Insertion
Tg(HDexon1)61Gpb expresses 1 gene
Mutation detailsA human HD fragment containing a polyglutamine-repeat expansion was isolated from a clone derived from a patient with Huntington's disease. The transgene contained approximately 1 kb of 5' UTR region, exon 1 which initially contained 113 CAG repeats, and 262 bp of intron 1 followed by a neomycin cassette. Subsequent analysis showed that the number of CAG repeats was prone to increase due to instability in the germline, and a range of 109.5 to 121 was observed. (J:36689)
View phenotypes and curated references for all genotypes (concatenated display).
Disease models
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Mouse strains and cell lines available from the International Mouse Strain Resource (IMSR)
Carrying this Mutation:  Mouse Strains: 3 strains available      Cell Lines: 0 lines available
The transgene is ubiquitously expressed.

Transgenic mice on a background that involves C57BL/6 and CBA display a progressive neurological phenotype that mimics many of the features of Huntington Disease in humans, including choreiform-like movements, involuntary stereotypic movements, tremor, and epileptic seizures, as well as nonmovement disorder components, including unusual vocalization. Frequent urination and loss of body weight and muscle bulk occurs through the course of the disease. Neurological developments include Neuronal Intranuclear Inclusions (NII), which contain both the huntingtin and ubiquitin proteins (NII have subsequently been identified in human HD patients). Onset of HD symptoms occurs between 15 and 21 weeks of age.

Original:  J:36689 Mangiarini L, et al., Exon 1 of the HD gene with an expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic mice. Cell. 1996 Nov 1;87(3):493-506
All:  151 reference(s)

Contributing Projects:
Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB)), Gene Ontology (GO)
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MGI 6.22
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