Mice have been generated that are transgenic for the 5' end of the human HD gene carrying (CAG)115-(CAG)150 repeat expansions. In this founder line (61Gpb), as well as another similar line (62Gpb, see Stock No. <a href="https://www.jax.org/strain/006494">006494</a>), the transgene is ubiquitously expressed. Transgenic mice exhibit a progressive neurological phenotype that mimics many of the features of HD, including choreiform-like movements, involuntary stereotypic movements, tremor, and epileptic seizures, as well as nonmovement disorder components, including unusual vocalization. They urinate frequently and exhibit loss of body weight and muscle bulk through the course of the disease. Neurologically they develop Neuronal Intranuclear Inclusions (NII) which contain both the huntingtin and ubiquitin proteins. These NII have also been identified in human HD patients. The age of onset of HD symptoms is reported to occur between 15 and 21 weeks for this 61Gpb line. These HDexon1 mice may be useful in Huntington's Disease research. In an attempt to offer alleles on well-characterized or multiple genetic backgrounds, alleles are frequently moved to a genetic background different from that on which an allele was first characterized. Mice with this mutation were originally published on a mixed CBAxC57BL6 genetic background. It should be noted that the phenotype could vary from that originally described. We will modify the strain description as published results become available. In an attempt to offer alleles on well-characterized or multiple genetic backgrounds, alleles are frequently moved to a genetic background different from that on which an allele was first characterized. Mice with this mutation were originally published on a mixed CBAxC57BL6 genetic background. It should be noted that the phenotype could vary from that originally described. We will modify the strain description as published results become available. 
 When maintaining the Stock No. 006471 live colony at The Jackson Laboratory, C57BL/6J inbred females (Stock No. <a href="https://www.jax.org/strain/000664">000664</a>) are bred to a hemizygous male (with CAG repeat range scoring as described below) in trio matings. It is our experience that this mating system produces approximately 3 litters before the hemizygote develops an HD-like phenotype, whereas the reciprocal mating (hemizygous females bred to C57BL/6J male) typically only produces 1 litter.
 Stock No. 006471 B6.Cg-Tg(HDexon1)61Gpb/J - Trinucleotide Scoring and Range [November 2024]:
 In 2024, The Jackson Laboratory scoring of this strain's trinucleotide (CAG) repeat values was changed to Transnetyx following their acquisition of Laragen. Subsequently, The Jackson Laboratory Genetic Quality Control group and Transnetyx performed side-by-side CAG repeat value analyses of the same hemizygous mouse samples (representing several different pedigrees within the live colony) via the Laragen protocol versus the Transnetyx protocol. For Stock No. 006471, ~50 mouse samples were analyzed - this showed the Laragen score averaging 1.18 trinucleotide repeats higher than the same sample scored via Transnetyx. Based on these analyses, The Jackson Laboratory has adjusted the trinucleotide score range we use for Stock No. 006471 hemizygous breeders from 115-118 CAG repeats via Laragen protocol to 114-117 CAG repeats via Transnetyx protocol [November 2024].

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R6/1 (HDexon1) transgenic mice exhibit a progressive neurological phenotype that mimics many of the features of Huntington's Disease and may be useful in research of that disease.

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