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Titolo:
Functional mitochondrial heterogeneity in heteroplasmic cells carrying themitochondrial DNA mutation associated with the MELAS syndrome (mitochondrial encephalopathy, lactic acidosis, and strokelike episodes)
Autore:
Bakker, A; Barthelemy, C; Frachon, P; Chateau, D; Sternberg, D; Mazat, JP; Lombes, A;
Indirizzi:
INSERM, UR523, Inst Myol, F-75651 Paris, France INSERM Paris France F-75651 ERM, UR523, Inst Myol, F-75651 Paris, France Hop La Salpetriere, F-75651 Paris, France Hop La Salpetriere Paris France F-75651 lpetriere, F-75651 Paris, France Univ Bordeaux 2, INSERM E9929, F-33076 Bordeaux, France Univ Bordeaux 2 Bordeaux France F-33076 E9929, F-33076 Bordeaux, France
Titolo Testata:
PEDIATRIC RESEARCH
fascicolo: 2, volume: 48, anno: 2000,
pagine: 143 - 150
SICI:
0031-3998(200008)48:2<143:FMHIHC>2.0.ZU;2-X
Fonte:
ISI
Lingua:
ENG
Soggetto:
CYTOCHROME-C-OXIDASE; PROTEIN-SYNTHESIS; MUTANT; DISEASE; MTDNA; COMPLEMENTATION; RESPIRATION; SEGREGATION; DEFICIENCY; GENETICS;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Life Sciences
Citazioni:
23
Recensione:
Indirizzi per estratti:
Indirizzo: Lombes, A Hop La Salpetriere, INSERM, UR 523, Inst Myol, F-75651 Paris 13,France Hop La Salpetriere Paris France 13 l, F-75651 Paris 13, France
Citazione:
A. Bakker et al., "Functional mitochondrial heterogeneity in heteroplasmic cells carrying themitochondrial DNA mutation associated with the MELAS syndrome (mitochondrial encephalopathy, lactic acidosis, and strokelike episodes)", PEDIAT RES, 48(2), 2000, pp. 143-150

Abstract

Most mitochondrial DNA (mtDNA) alterations associated with human disordersare heteroplasmic, i.e. mutant mtDNA molecules coexist with normal ones within the cell. We addressed the possibility of intermitochondrial exchangesthrough histologic analyses of cybrid clones with increasing proportion ofthe MELAS (A3243G) mtDNA transfer RNA point mutation. MtDNA-dependent cytochrome c oxidase activity and protein composition as well as mitochondrial membrane potential appeared heterogeneous in individual cells from clonal heteroplasmic cell populations on the basis of confocal and electron microscopy. The number of defective cells increased with increasing mutation load. We conclude that in the presence of a heteroplasmic mtDNA mutation in the cell type that we studied, intermitochondrial molecular exchanges cannot provide an efficient even distribution of the complementing molecules such aswild-type mtDNA, transfer RNA, or protein. Mitochondria in these heteroplasmic cells cannot, therefore, be considered a single functional unit.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 19/01/20 alle ore 20:21:44