B6C3-Tg(HD82Gln)81Gschi/J

Stock number: 003627
Product name: N171-82Q mice

Description

Under control of a mouse prion protein promoter, which drives transgene expression in the neurons of the central nervous system, these transgenic mice express an N-terminally truncated human Huntingtin cDNA. They develop behavioral abnormalities, including loss of coordination, tremors, hypokinesis and abnormal gait, before dying prematurely.

Detailed description

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.

Allele

Symbol: Tg(HD82Gln)81Gschi
Name: transgene insertion 81, Gabriele Schilling
MGI accession ID: MGI:2670626
Generation method: Transgenic
Attributes: Inserted expressed sequence; Humanized sequence
Marker symbol: Tg(HD82Gln)81Gschi
Marker name: transgene insertion 81, Gabriele Schilling
Chromosome: UN
Strain of origin: C3H/B6
Expressed genes: HTT,huntingtin,human
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