Mitochondrial energy failure in HSD10 disease is due to defective mtDNA transcript processing

Muscle, heart and liver were analyzed in a male subject who succumbed to HSD10 disease. Respiratory chain enzyme analysis and BN-PAGE showed reduced activities and assembly of complexes I, III, IV, and V. The mRNAs of all RNase P subunits were preserved in heart and overexpressed in muscle, but MRPP...

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Detalhes bibliográficos
Publicado no:Mitochondrion
Main Authors: Chatfield, Kathryn C., Coughlin, Curtis R., Friederich, Marisa W., Gallagher, Renata C., Hesselberth, Jay R., Lovell, Mark A., Ofman, Rob, Swanson, Michael A., Thomas, Janet A., Wanders, Ronald J.A., Wartchow, Eric P., Van Hove, Johan L.K.
Formato: Artigo
Idioma:Inglês
Publicado em: 2015
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Article
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC4355277/
https://ncbi.nlm.nih.gov/pubmed/25575635
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1016/j.mito.2014.12.005
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