Phenotypes Associated with This Genotype

Genotype
MGI:3767789

• Allelic Composition: Tg(CAG-AR*97Q)7-8Sobue/?
• Genetic Background: involves: C57BL/6 * C57BL/6J * DBA/2

• ♀: phenotype observed in females
• ♂: phenotype observed in males
• N: normal phenotype

mortality/aging

premature death ( J:128619 )

• life spans are reduced to 66 to 132 days for male mice from cachexia due to hyponutrition and dehydration

• however, female mice survive past 210 days

• female life span is reduced when treated with testoserone where as male life span is increased following castration

behavior/neurological

impaired coordination ( J:121559 , J:128619 )

• male mice display impaired motor coordination on a rotarod test at 8 weeks of age (J:121559)

• male mice take short steps or drag their feet (J:121559)

• progressive motor impairment as assessed by a rotarod test begins at 8 to 9 weeks of age for male mice and at 15 to 21 weeks for female mice (J:128619)

• female motor coordination is decreased when treated with testosterone whereas males motor coordination improve following castration (J:128619)

decreased locomotor activity ( J:121559 , J:128619 )

• male mice become hypoactive at 32 weeks of age (J:121559)

• male mice display pronounced and accelerated reduction in cage activity compared to female mice (J:128619)

• hypoactivity is increased in female mice following treatment with testosterone (J:128619)

nervous system

abnormal motor neuron morphology ( J:128619 )

• motor neuron cross-sectional area is decreased (130.6+/-4.0 um² compared to 195.6+/-12.1 um² in wild-type mice)

growth/size/body

decreased body size ( J:128619 )

• male mice display pronounced and accelerated reduction in body size compared to female mice

weight loss ( J:121559 , J:128619 )

• male mice lose more weight earlier than Tg(ACTB-STUB1)Sobu/Tg(ACTB-STUB1)Sobu Tg(ACTB-AR*97Q)2-6Sobu mice and Tg(ACTB-STUB1)Sobu Tg(ACTB-AR*97Q)2-6Sobu mice (J:121559)

• weight lose begins at 4 to 6 weeks for male mice and at 12 to 16 weeks for female mice (J:128619)

• weight loss in females is accelerated by treatment with testosterone and decelerated in castrated males (J:128619)

cachexia ( J:128619 )

• male mice die due to cachaxia

muscle

dystrophic muscle ( J:128619 )

• male mice display pronounced and accelerated muscular dystrophy compared to female mice

• male mice display muscular atrophy in the trunk and hindlimbs followed by weight loss and impaired motor coordination

• female muscular dystrophy is worsened by treatment with testosterone whereas it is improved in castrated male mice

muscular atrophy ( J:121559 )

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
Kennedy's disease		DOID:0060161	OMIM:313200	J:128519