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Analysis of the mitochondrial proteome of cybrid cells harbouring a truncative mitochondrial DNA mutation in respiratory complex I.

Molecular bioSystems - 1 Jun 2014

Musicco Clara, Cormio Antonella, Calvaruso Maria Antonietta, Iommarini Luisa, Gasparre Giuseppe, Porcelli Anna Maria, Timperio Anna Maria, Zolla Lello, Gadaleta Maria Nicola

Abstract excerpt

Transmitochondrial cytoplasmic hybrids (cybrids) are well established model systems to reveal the effects of mitochondrial DNA (mtDNA) mutations on cell metabolism excluding the interferences of a different nuclear background. The m.3571insC mutation in the MTND1 gene of respiratory complex I (CI) is commonly detected in oncocytic tumors, in which it causes a severe CI dysfunction leading to an energetic...

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PMID: 24643264
DOI: 10.1039/c3mb70542k

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LDHB

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