Phenotypes Associated with This Genotype

Genotype
MGI:5897176

• Allelic Composition: Tg(CAG-Ppard*E411P)#Als/0; Tg(Nes-cre)1Kln/0
• Genetic Background: B6J.Cg-Tg(Nes-cre)1Kln Tg(CAG-Ppard*E411P)#Als

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

growth/size/body

decreased body weight ( J:233176 )

• mice appear smaller by 4-6 months of age and weigh less than controls by 8 months of age

behavior/neurological

decreased exploration in new environment ( J:233176 )

• mice exhibit reduced novel-environment exploration

abnormal response to novel object ( J:233176 )

• mice exhibit reduced novel-object recognition

abnormal motor capabilities/coordination/movement ( J:233176 )

• mice exhibit prominent motor abnormalities by 8 months of age

• in the cage-ledge test, mice cannot easily dismount from the cage ledge

limb grasping ( J:233176 )

• mice exhibit a prominent clasping phenotype

impaired coordination ( J:233176 )

• mice exhibit worse latency-to-fall times on the accelerating rotarod

decreased grip strength ( J:233176 )

• reduction in combined mesh and grip-strength

short stride length ( J:233176 )

• decrease in mean stride-length

cellular

decreased mitochondrial size ( J:233176 )

• mitochondria in the striatum and cortex are smaller in size

abnormal mitochondrial ATP synthesis coupled electron transport ( J:233176 )

• activities of mitochondrial complex IV and complex V are reduced in the striatum and overall ATP concentration is reduced in the striatum and cortex

nervous system

decreased brain size ( J:233176 )

• brain size of adults is reduced

abnormal basal ganglion morphology ( J:233176 )

• reduction in the volume of the basal ganglia

abnormal cerebral cortex morphology ( J:233176 )

• reduction in the volume of the cortex

gliosis ( J:233176 )

• reactive gliosis in the brain

decreased neuron number ( J:233176 )

• brain shows reduction in neuron numbers

decreased dopaminergic neuron number ( J:233176 )

• reduction in tyrosine hydroxylase-positive dopaminergic neurons in the substantia nigra

neurodegeneration ( J:233176 )

skeleton

kyphosis ( J:233176 )

Mouse Models of Human Disease		DO ID	OMIM ID(s)	Ref(s)
Huntington's disease		DOID:12858	OMIM:143100	J:233176