Novel mitochondrial protein kinase involved in early onset Parkinson’s disease

A newly discovered protein called PINK1 has been found to be associated with Parkinson’s disease. This protein kinase was shown to localize to mitochondria. The recently identified mutation in this protein (G309D) was shown to greatly increase mitochondrial dysfunction and apoptosis. See Valeute et al, Science 304 1158-1160 (2004). Note that this work used a Complex I monoclonal antibody as a molecular marker available from MitoSciences for the mitochondrial fraction.

Aging results from accumulated mutations in mitochondrial DNA

A homozygous knock in mouse has been developed that expresses a mitochondrial polymerase that is defective in proof-reading function. Such mice were found to have a 3-5 fold increase in the levels of point mutations and increased amounts of deleted mt-DNA which both reduced lifespan and caused premature onset of ageing including weight loss, hair loss, osteoporosis and reduced fertility. See Trifunovic et al, Nature 429 417-423 (2004). Note that this work used a cytochrome oxidase (COX) II antibody available from MitoSciences to monitor mitochondrial protein synthesis.