Phenotypes for Htt Ppargc1a MGI:3698750 MGI Mouse

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Phenotypes Associated with This Genotype

Allelic Composition	Htt^tm1Mfc/Htt⁺ Ppargc1a^tm1Dpk/Ppargc1a⁺
Genetic Background	involves: 129S1/Sv * 129X1/SvJ

Find Mice

Using the International Mouse Strain Resource (IMSR)
Mouse lines carrying:
Htt^tm1Mfc mutation (2 available); any Htt mutation (178 available)
Ppargc1a^tm1Dpk mutation (1 available); any Ppargc1a mutation (48 available)

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

behavior/neurological

impaired coordination ( J:116058 )

• rotarod performance is severely impaired and much worse than in mice carrying the Hdh^tm1Mfc knock-in allele

nervous system

abnormal striatum morphology ( J:116058 )

• striatal neuronal volumes are significantly decreased in 6-month old mutants compared to mice carrying the Hdh^tm1Mfc knock-in allele

neuron degeneration ( J:116058 )

• early neuronal degeneration appears in the striatum and the medial septal nucleus by 3 months of age

• mutants exhibit increased susceptibility to 3-nitropropionic acid, developing larger striatal lesions and increased number of degenerating neurons compared to mice carrying the Hdh^tm1Mfc knock-in allele

Contributing Projects: 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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