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Ziegler 2012 Mitochondrion

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Publications in the MiPMap
Ziegler CG, Peng M, Falk MJ, Polyak E, Tsika E, Ischiropoulos H, Bakalar D, Blendy JA, Gasser DL (2012) Parkinson's disease-like neuromuscular defects occur in prenyl diphosphate synthase subunit 2 (Pdss2) mutant mice. Mitochondrion 12:248-57.

Β» PMID: 21983691 Open Access

Ziegler CG, Peng M, Falk MJ, Polyak E, Tsika E, Ischiropoulos H, Bakalar D, Blendy JA, Gasser DL (2012) Mitochondrion

Abstract: The Pdss2 gene product is needed for the isoprenylation of benzoquinone to generate coenzyme Q (CoQ). A fatal kidney disease occurs in mice that are homozygous for a missense mutation in Pdss2, which can be recapitulated in conditional Pdss2 knockouts targeted to glomerular podocytes. We now report that homozygous missense mutants also demonstrate significant neuromuscular deficits, as validated by behavioral and coordination assays, and these deficits are recapitulated in conditional Pdss2 knockouts targeted to dopaminergic neurons. Both conditional knockout and missense mutant mice demonstrate deficiencies in tyrosine hydroxylase-positive neurons in the substantia nigra, implicating a pathology similar to sporadic Parkinson's disease (PD). β€’ Keywords: Parkinson's disease, Pdss2 knockout

β€’ O2k-Network Lab: US PA Philadelphia Falk MJ


Labels: MiParea: Respiration, Genetic knockout;overexpression  Pathology: Parkinson's 

Organism: Mouse  Tissue;cell: Nervous system  Preparation: Permeabilized tissue 



HRR: Oxygraph-2k