Analysis Tools
mortality/aging
| N |
• expression of the transgene rescues the embryonic lethality observed in Hdh-null animals
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Allele Symbol Allele Name Allele ID |
Tg(YAC128)53Hay transgene insertion 53, Michael Hayden MGI:3613515 |
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| Summary |
4 genotypes
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
| N |
• expression of the transgene rescues the embryonic lethality observed in Hdh-null animals
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
| N |
• mice do not exhibit a deterioration in grip strength as compared to controls
• stride length, splay length and base are similar to controls
• rearing activity is similar to controls
• mice do not behave differently from controls in the light/dark choice test
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• mice do not perform as well on rotarod test as controls, but performance does not worsen with age
• females perform worse than males
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• mice are hypoactive (total distance covered) in the open field test starting at 12 weeks of age in the light phase and 8,16 and 34 weeks in the dark phase
• hypoactivity in the center is only observed in the dark phase at 16 and 34 weeks
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• body weight is significantly increased in males at 8 weeks and females at 16 weeks, although male weight does not reach significance at 56 weeks
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| N |
• mice do not exhibit alterations in startle reflex although there is significant variability across age and gender
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• observed at 56 weeks of age
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
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• in medium spiny neurons in response to NMDA (500 um) exposure compared to wild-type mice or mice carrying Tg(YAC128)55Hay
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• 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)
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• mean area of striatal neurons is decreased by 18% in 12 month old mice
• striatal volume is decreased by 15% by 9 months of age
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• 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)
• 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)
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• medium spiny neurons (MSN) are decreased 8% by 9 months of age
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• transgenic mice exhibit a 9% decrease in striatal neurons by 9 months, progressing to a 15-18% loss by 12 months
• medium spiny neurons (MSN), the major neuronal cell type of the straitum, are decreased 8% by 9 months of age
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• 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)
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• 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)
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• cognitive deficits including difficulties in changing strategies and delayed platform finding beginning at 2 months of age
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• impaired in a rotarod assay beginning at 2 months of age
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• motor abnormalities similar to those seen in the clinical course of Huntington disease
• disease progression is accelerated compared to mice hemizygous for Tg(YAC128)55Hay
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• 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)
• significant deficit in rotarod tests at 2 months of age
(J:120991)
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• 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)
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• hyperkinetic phenotype observed at 3 months in open field apparatus
(J:84453)
• at 2 months of age
(J:105723)
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• starting at 4 months of age, a hypokinetic phenotype is displayed compared to wild-type controls in open field test
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• in medium spiny neurons in response to NMDA (500 um) exposure compared to wild-type mice or mice carrying Tg(YAC128)55Hay
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• in medium spiny neurons in response to NMDA (500 um) exposure compared to wild-type mice or mice carrying Tg(YAC128)55Hay
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Mouse Models of Human Disease |
DO ID | OMIM ID(s) | Ref(s) | |
| Huntington's disease | DOID:12858 |
OMIM:143100 |
J:84453 , J:105723 , J:111237 | |
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| ♀ | phenotype observed in females |
| ♂ | phenotype observed in males |
| N | normal phenotype |
| N |
• mice do not exhibit a deterioration in grip strength as compared to controls
• stride length, splay length and base are similar to controls
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• increased preference for dark in males in dark/light choice test starting at 52 weeks of age
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• mice do not perform as well on rotarod test as controls, but performance does not worsen with age
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• mice exhibit a decrease in climbing activity at 16 weeks of age
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• mice are hypoactive (total distance covered) in dark phase of open field test starting at 36 and 52 weeks of age in females and 16 weeks of age in males
• hypoactivity is not observed in light phase
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• in the light phase of the open field test, females are hyperactive as compared to controls only at 52 weeks of age
• in the dark phase, mice cover more distance in center than controls
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• mice rear more at 36 and 52 weeks in the light phase of the open field test
• in the dark phase males rear more only at 52 weeks of age
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• body weight is significantly increased in males at 32 weeks
• females are not consistently heavier, exhibiting heavier weights between 48 and 76 weeks
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| N |
• mice do not exhibit alterations in startle reflex or in prepulse inhibition
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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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last database update 04/16/2024 MGI 6.23 |
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