Mitochondrial Respiratory Dysfunction in Familiar Parkinsonism Associated with PINK1 Mutation (Articolo in rivista)

Type

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

Label

• Mitochondrial Respiratory Dysfunction in Familiar Parkinsonism Associated with PINK1 Mutation (Articolo in rivista) (literal)

Anno

• 2008-01-01T00:00:00+01:00 (literal)

Alternative label

• Piccoli C, Sardanelli A, Scrima R, Ripoli M, Quarato G, D'Aprile A, Bellomo F,Scacco S, De Michele G, Filla A, Iuso A, Boffoli D, Capitanio N, Papa S. (2008)
  Mitochondrial Respiratory Dysfunction in Familiar Parkinsonism Associated with PINK1 Mutation
  in Neurochemical research
  (literal)

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

• Piccoli C, Sardanelli A, Scrima R, Ripoli M, Quarato G, D'Aprile A, Bellomo F,Scacco S, De Michele G, Filla A, Iuso A, Boffoli D, Capitanio N, Papa S. (literal)

Rivista

• Neurochemical research (Rivista)

Note

• ISI Web of Science (WOS) (literal)

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

• Department of Biomedical Sciences, University of Foggia,Department of Medical Biochemistry, Biology and Physics,University of Bari, Piazza G. Cesare, 70100 Bari, Italy, Department of Neurological Sciences, Federico II University,Institute of Biomembranes and Bioenergetics, Italian ResearchCouncil, Bari, Italy (literal)

Titolo

• Mitochondrial Respiratory Dysfunction in Familiar Parkinsonism Associated with PINK1 Mutation (literal)

Abstract

• In the present study mitochondrial respiratory function of fibroblasts from a patient affected by early-onset Parkinsonism carrying the homozygous W437X nonsense mutation in the PINK1 gene has been thoroughly characterized. When compared with normal fibroblasts, the patient's fibroblast mitochondria exhibited a lower respiratory activity and a decreased respiratory control ratio with cellular ATP supply relying mainly on enhanced glycolytic production. The quantity, specific activity and subunit pattern of the oxidative phosphorylation complexes were normal. However, a significant decrease of the cellular cytochrome c content was observed and this correlated with a reduced cytochrome c oxidase in situ-activity. Measurement of ROS revealed in mitochondria of the patient's fibroblasts enhanced O (2) (*-) and H(2)O(2) production abrogated by inhibition of complex I. No change in the glutathione-based redox buffering was, however, observed. (literal)

Prodotto di

• Sistemi boenergetici di membrana: meccanismi funzionali e fisiopatologia. (SV.P14.005.001) (Modulo)
• Institute of biomembrane and bioenergetics (IBBE) (Istituto)

Autore CNR

• SERGIO PAPA (Unità di personale esterno)

Incoming links:

Prodotto

• Institute of biomembrane and bioenergetics (IBBE) (Istituto)
• Sistemi boenergetici di membrana: meccanismi funzionali e fisiopatologia. (SV.P14.005.001) (Modulo)

Autore CNR di

• SERGIO PAPA (Unità di personale esterno)

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

• Neurochemical research (Rivista)