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Strain

B6.129S4-Prkn^tm1Shn/J

Summary

Data Summary

Official Name	B6.129S4-Prkn^tm1Shn/J
Common Name	PARK2 KO
Description	Homozygous Parkinson disease (autosomal recessive, juvenile) 2, parkin (Park2) knock-out mice have increased extracellular dopamine concentration in the striatum, dysfunctional nigrostriatal pathways, and dysfunctional mitochondria. These mice model the exon 3 deletion mutation most common in human autosomal recessive juvenile parkinsonism patients and may be useful in studies of Parkinson's disease, dopamine regulation, nigrostriatal function, mitochondrial function, and other neurobiological research.
Development Status	Phenotyping ongoing
Creation Method	knockout
Breeding Type	intercross
Phenotype Description	Homozygous mice are viable and fertile, and exhibit grossly normal brain morphology. Western blot analysis using antibody specific to C-terminal sequences indicates the absence of full length gene product. RT-PCR shows that exon 2 splices to exon 4, skipping exon 3 entirely, resulting in a frame shift and a premature stop codon in exon 5. While EGFP transcripts are present, little parkin-EGFP fusion protein is detectable by Western analysis. Homozygous mice have increased extracellular dopamine concentration in the striatum. Further, medium-sized striatal spiny neurons require greater currents to induce synaptic responses, suggesting a reduction in synaptic excitability in the absence of the endogenous gene. Homozygotes also exhibit deficits in behavioral paradigms sensitive to dysfunction of the nigrostriatal pathway. The numbers of dopaminergic neurons in the substantia nigra, however, are normal up to the age of 24 months, in contrast to the substantial loss of nigral neurons characteristic of Parkinson's disease. Homozygous mice and their isolated cells exhibit mitochondrial dysfunction and impaired protection from oxidative stress. Muscle cells isolated from homozygous mice have defective skeletal muscle mitochondrial homeostasis and increased sensitivity to amyloid-beta toxicity. These mice model the exon 3 deleti on mutation most common in human autosomal recessive juvenile parkinsonism (AR-JP) patients and may be useful in studies of Parkinson's disease, dopamine regulation, nigrostriatal function, mitochondrial function, and other neurobiological research.

Type	Count
Investigators	1
Genomics - Modifications	1
Experiments	7
Publications	1

Investigators

Name	Institution
Michael Jurczak	University of Pittsburgh School of Medicine

Genomic Information

Gene	Allele 1	Allele 2	Protocol
Park2	knockout	knockout	Not Specified

OrderID	Order	Investigator	Experiment	Status	Measurements
22426	22426	Callie Corsa	2017-11-07 MacDougald - Call 16 Mice NMR	Completed	192
22426	22426	Callie Corsa	2017-11-07 MacDougald - Callie 16 Mice 3 Day CLAMS Run	Completed	6816
22434	22434	Callie Corsa	2017-11-13 MacDougald - Callie 15 Mice 3 Day CLAMS Run	Completed	6360
22434	22434	Callie Corsa	2017-11-13 MacDougald - Callie 15 Mice NMR	Completed	180
21945	21945	Michael Jurczak	PARK2 male chow clamp	Completed	130
21945	21945	Michael Jurczak	PARK2 male chow CLAMS	Completed	275
21945	21945	Michael Jurczak	PARK2 male chow to HFD CLAMS	Completed	165

Publication	Altmetrics	Submitted By	PubMed ID	Status
Reduced intestinal lipid absorption and body weight-independent improvements in insulin sensitivity in high-fat diet-fed Park2 knockout mice. Costa DK, Huckestein BR, Edmunds LR, Petersen MC, Nasiri A, Butrico GM, Abulizi A, Harmon DB, Lu C, Mantell BS, Hartman DJ, Camporez JP, O'Doherty RM, Cline GW, Shulman GI, Jurczak MJ American journal of physiology. Endocrinology and metabolism, 2016 (311), E105 - 16		Submitted Externally	27166280	Published

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