Phenotypes Associated with This Genotype

Genotype
MGI:6489606

• Allelic Composition: Ppargc1a^tm1Dpk/Ppargc1a^tm1Dpk
• Genetic Background: B6.129X1-Ppargc1a^tm1Dpk/J

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

behavior/neurological

impaired coordination ( J:298664 )

• motor coordination is severely impaired in middle-aged (10 months of age) mice and even further in old (20 months of age) mice on the accelerating rotarod

decreased locomotor activity ( J:298664 )

• a group of 10-month old and 20-month old mice show fewer spontaneous, voluntary movements in the open field

bradykinesia ( J:298664 )

• old mice at 20 months of age exhibit significant bradykinesia during pole testing

homeostasis/metabolism

decreased nervous system dopamine level ( J:298664 )

• levels of dopamine and its metabolites (DOPAC and HVA) in the striatum are reduced in 10-month old mice and are even further reduced in 20-month old mice

nervous system

abnormal substantia nigra morphology ( J:298664 )

• mice exhibit reduced expression of mitochondrial markers in the substantia nigra

neurodegeneration ( J:298664 )

• mice exhibit age-related dopaminergic neurodegeneration, with a significant reduction in TH+ neurons at 20 months of age

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
Parkinson's disease		DOID:14330	OMIM:PS168600	J:298664