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Phenotypes Associated with This Genotype
Genotype
MGI:3613525
Allelic
Composition
Tg(YAC128)53Hay/0
Genetic
Background
FVB/N-Tg(YAC128)53Hay
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Mouse lines carrying:
Tg(YAC128)53Hay mutation (1 available)
phenotype observed in females
phenotype observed in males
N normal phenotype
nervous system
• in medium spiny neurons in response to NMDA (500 um) exposure compared to wild-type mice or mice carrying Tg(YAC128)55Hay (J:105723)
• in medium spiny neurons in response to NMDA (500 um) exposure compared to wild-type mice or mice carrying Tg(YAC128)55Hay (J:105723)
• total brain weight decreases by 5% at 9 months, progressing to a 10% decrease by 1 year (J:84453)
• cerebellum weight is unchanged (J:84453)
• total brain weight decreases by 5% at 9 months, progressing to a 10% decrease by 1 year (J:84453)
• cerebellum weight is unchanged (J:84453)
• reduced compared to wild-type controls at 18 months (0.35 grams vs 0.38 grams) (J:120991)
• reduced compared to wild-type controls at 18 months (0.35 grams vs 0.38 grams) (J:120991)
• mean area of striatal neurons is decreased by 18% in 12 month old mice (J:84453)
• striatal volume is decreased by 15% by 9 months of age (J:84453)
• mean area of striatal neurons is decreased by 18% in 12 month old mice (J:84453)
• striatal volume is decreased by 15% by 9 months of age (J:84453)
• loss of striatal neurons (J:105723)
• decrease in striatal volume at 12 months of age (J:105723)
• loss of striatal neurons (J:105723)
• decrease in striatal volume at 12 months of age (J:105723)
• the levels of both the endogenous excitotoxin quinolinic acid (QUIN) and its bioprecursor, 3-hydroxykynurenine (3-HK) are increased in the striatum beginning at 8 months of age, similarly to that seen in Huntington disease patients (J:111237)
• the levels of both the endogenous excitotoxin quinolinic acid (QUIN) and its bioprecursor, 3-hydroxykynurenine (3-HK) are increased in the striatum beginning at 8 months of age, similarly to that seen in Huntington disease patients (J:111237)
• reduced compared to wild-type controls at 18 months (10.9 mm3 vs 12.3 mm3) (J:120991)
• significant volume loss is detected at 12 months compared to wild-type controls (J:120991)
• neuronal loss is observed at 12 months relative to controls (J:120991)
• diffuse huntingtin (htt) fragments showing nuclear localization in striatum at 3 months, and this increases with age such that at 12 months, most striatal neurons are positive for htt (J:120991)
• reduced compared to wild-type controls at 18 months (10.9 mm3 vs 12.3 mm3) (J:120991)
• significant volume loss is detected at 12 months compared to wild-type controls (J:120991)
• neuronal loss is observed at 12 months relative to controls (J:120991)
• diffuse huntingtin (htt) fragments showing nuclear localization in striatum at 3 months, and this increases with age such that at 12 months, most striatal neurons are positive for htt (J:120991)
• medium spiny neurons (MSN) are decreased 8% by 9 months of age (J:84453)
• medium spiny neurons (MSN) are decreased 8% by 9 months of age (J:84453)
• transgenic mice exhibit a 9% decrease in striatal neurons by 9 months, progressing to a 15-18% loss by 12 months (J:84453)
• medium spiny neurons (MSN), the major neuronal cell type of the straitum, are decreased 8% by 9 months of age (J:84453)
• transgenic mice exhibit a 9% decrease in striatal neurons by 9 months, progressing to a 15-18% loss by 12 months (J:84453)
• medium spiny neurons (MSN), the major neuronal cell type of the straitum, are decreased 8% by 9 months of age (J:84453)
• cortex volume decreased by 7% by 12 months of age (J:84453)
• cortex volume decreased by 7% by 12 months of age (J:84453)
• QUIN and 3-HK levels are elevated in the cerebral cortex, similarly to that seen in Huntington disease patients (J:111237)
• QUIN and 3-HK levels are elevated in the cerebral cortex, similarly to that seen in Huntington disease patients (J:111237)
• neuronal inclusions observed throughout nucleoplasm of all striatal cells by 18 months of age (J:84453)
• neuronal inclusions observed throughout nucleoplasm of all striatal cells by 18 months of age (J:84453)
• at 18 months of age in the striatum and cortex (J:105723)
• at 18 months of age in the striatum and cortex (J:105723)

behavior/neurological
• cognitive deficits including difficulties in changing strategies and delayed platform finding beginning at 2 months of age (J:105723)
• cognitive deficits including difficulties in changing strategies and delayed platform finding beginning at 2 months of age (J:105723)
• impaired in a rotarod assay beginning at 2 months of age (J:105723)
• impaired in a rotarod assay beginning at 2 months of age (J:105723)
• motor abnormalities similar to those seen in the clinical course of Huntington disease (J:105723)
• disease progression is accelerated compared to mice hemizygous for Tg(YAC128)55Hay (J:105723)
• motor abnormalities similar to those seen in the clinical course of Huntington disease (J:105723)
• disease progression is accelerated compared to mice hemizygous for Tg(YAC128)55Hay (J:105723)
• progressive decrease in fixed speed rotarod performance beginning at 6 months of age (J:84453)
• progressive decrease in fixed speed rotarod performance beginning at 6 months of age (J:84453)
• in a rotarod assay beginning after 3 months of age and becoming worse with age (J:105723)
• in a rotarod assay beginning after 3 months of age and becoming worse with age (J:105723)
• significant deficit in rotarod tests at 2 months of age (J:120991)
• significant deficit in rotarod tests at 2 months of age (J:120991)
• starting at 4 months of age, a hypokinetic phenotype is displayed compared to wild-type controls in open field test (J:120991)
• starting at 4 months of age, a hypokinetic phenotype is displayed compared to wild-type controls in open field test (J:120991)
• hyperkinetic phenotype observed at 3 months in open field apparatus (J:84453)
• hyperkinetic phenotype observed at 3 months in open field apparatus (J:84453)
• at 2 months of age (J:105723)
• at 2 months of age (J:105723)
• hypokinetic phenotype observed beginning at 6 months of age and becoming significant by 12 months of age in open field apparatus (J:84453)
• hypokinetic phenotype observed beginning at 6 months of age and becoming significant by 12 months of age in open field apparatus (J:84453)
• beginning after 3 months of age as measured by decrease in spontaneous ambulation in open-field testing (J:105723)
• beginning after 3 months of age as measured by decrease in spontaneous ambulation in open-field testing (J:105723)

growth/size/body

cellular
• in medium spiny neurons in response to NMDA (500 um) exposure compared to wild-type mice or mice carrying Tg(YAC128)55Hay (J:105723)
• in medium spiny neurons in response to NMDA (500 um) exposure compared to wild-type mice or mice carrying Tg(YAC128)55Hay (J:105723)

homeostasis/metabolism
• in medium spiny neurons in response to NMDA (500 um) exposure compared to wild-type mice or mice carrying Tg(YAC128)55Hay (J:105723)
• in medium spiny neurons in response to NMDA (500 um) exposure compared to wild-type mice or mice carrying Tg(YAC128)55Hay (J:105723)

Mouse Models of Human Disease
OMIM ID Ref(s)
Huntington Disease; HD 143100 J:84453 , J:105723 , J:111237


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Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Tumor Biology (MTB), Gene Ontology (GO), MouseCyc
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last database update
02/02/2016
MGI 6.02
The Jackson Laboratory