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Genes Cells ; 21(7): 772-88, 2016 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-27270837

RESUMO

DJ-1 has been identified as a gene responsible for recessive familial Parkinson's disease (familial Parkinsonism), which is caused by a mutation in the PARK7 locus. Consistent with the inferred correlation between Parkinson's disease and mitochondrial impairment, mitochondrial localization of DJ-1 and its implied role in mitochondrial quality control have been reported. However, the mechanism by which DJ-1 affects mitochondrial function remains poorly defined, and the mitochondrial localization of DJ-1 is still controversial. Here, we show the mitochondrial matrix localization of various pathogenic and artificial DJ-1 mutants by multiple independent experimental approaches including cellular fractionation, proteinase K protection assays, and specific immunocytochemistry. Localization of various DJ-1 mutants to the matrix is dependent on the membrane potential and translocase activity in both the outer and the inner membranes. Nevertheless, DJ-1 possesses neither an amino-terminal alpha-helix nor a predictable matrix-targeting signal, and a post-translocation processing-derived molecular weight change is not observed. In fact, wild-type DJ-1 does not show any evidence of mitochondrial localization at all. Such a mode of matrix localization of DJ-1 is difficult to explain by conventional mechanisms and implies a unique matrix import mechanism for DJ-1 mutants.


Assuntos
Potencial da Membrana Mitocondrial/genética , Proteínas Mutantes/genética , Doença de Parkinson/genética , Proteína Desglicase DJ-1/genética , Humanos , Mitocôndrias/genética , Membranas Mitocondriais/química , Proteínas Mutantes/isolamento & purificação , Mutação , Doença de Parkinson/patologia , Proteína Desglicase DJ-1/química , Proteína Desglicase DJ-1/isolamento & purificação
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