http://www.cnr.it/ontology/cnr/individuo/prodotto/ID279442

Analysis of Mitochondrial Proteome of Cybrid Cells Harbouring a Truncative Mitochondrial DNA Mutation in Respiratory Complex I. (Articolo in rivista)

Type

Prodotto della ricerca (Classe)
Articolo in rivista (Classe)

Label

Analysis of Mitochondrial Proteome of Cybrid Cells Harbouring a Truncative Mitochondrial DNA Mutation in Respiratory Complex I. (Articolo in rivista) (literal)

Anno

2014-01-01T00:00:00+01:00 (literal)

Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#doi

10.1039/C3MB70542K (literal)

Alternative label

Clara Musicco,+*a Antonella Cormio,b Maria Antonietta Calvaruso,c Luisa Iommarini,c Giuseppe Gasparre,d Anna Maria Porcelli,ce Anna Maria Timperio,f Lello Zolla,f and Maria Nicola Gadaleta,ab (2014)
Analysis of Mitochondrial Proteome of Cybrid Cells Harbouring a Truncative Mitochondrial DNA Mutation in Respiratory Complex I.
in Molecular bioSystems (Print); Royal Society of Chemistry, Cambridge (Regno Unito)
(literal)

Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#autori

Clara Musicco,+*a Antonella Cormio,b Maria Antonietta Calvaruso,c Luisa Iommarini,c Giuseppe Gasparre,d Anna Maria Porcelli,ce Anna Maria Timperio,f Lello Zolla,f and Maria Nicola Gadaleta,ab (literal)

Rivista

Molecular bioSystems (Rivista)

Note

ISI Web of Science (WOS) (literal)
Scopus (literal)

Http://www.cnr.it/ontology/cnr/pubblicazioni.owl#affiliazioni

a CNR - Institute of Biomembranes and Bioenergetics, Bari, Italy. b Department of Biosciences, Biotechnologies and Biopharmaceutics, University of Bari, Italy c Department of Pharmacy and Biotechnology (FABIT), University of Bologna, Italy d Department of Medical and Surgical Sciences, Unit of Medical Genetics, University of Bologna, Italy e Centro Interdipartimentale di Ricerca Industriale Scienze della Vita e Tecnologie per la Salute, University of Bologna, Italy f Department of Ecological and Biological Sciences, Tuscia University, Viterbo, Italy (literal)

Titolo

Analysis of Mitochondrial Proteome of Cybrid Cells Harbouring a Truncative Mitochondrial DNA Mutation in Respiratory Complex I. (literal)

Abstract

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 impairment when present above 83% mutant load. To assess whether the energetic deficit may alter the mitochondrial proteome, OS-78 and OS-93 cybrid cell lines bearing two different degrees of the m.3571insC mutation (78% and 92.8%, respectively) and control cybrids bearing wild-type mtDNA (CC) were analyzed. Twodimensional electrophoresis and mass spectrometry revealed significant alterations only in cybrids above the threshold (OS-93). All differentially expressed proteins are decreased. In particular, the levels of the pyruvate dehydrogenase E1 chain B subunit (E1b), of lipoamide dehydrogenase (E3), the enzyme component of pyruvate and 2-oxoglutarate dehydrogenase complexes, and of lactate dehydrogenase B (LDHB) were reduced. Moreover, a significant decrease of the pyruvate dehydrogenase complex activity was found when OS-93 cybrid cells were grown in galactose medium, a metabolic condition that forces cells to use respiration. These results demonstrate that the energetic impairment caused by the almost homoplasmic m.3571insC mutation perturbs cellular metabolism leading to a decreased steady state level of components of very important mitochondrial NAD-dependent dehydrogenases. (literal)

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