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Titolo:
Mitochondrial expression of a short dystrophin-like product with molecularweight of 71 kDa
Autore:
Chavez, O; Harricane, MC; Aleman, V; Dorbani, L; Larroque, C; Mornet, D; Rendon, A; Martinez-Rojas, D;
Indirizzi:
IPN, CINVESTAV, Dept Physiol Biophys & Neurosci, Mexico City, DF, Mexico IPN Mexico City DF Mexico ol Biophys & Neurosci, Mexico City, DF, Mexico Univ Montpellier 1, CNRS CRBM UPR 1086, F-34293 Montpellier 5, France UnivMontpellier 1 Montpellier France 5 6, F-34293 Montpellier 5, France Univ Sci & Technol, Neurobiol Lab, Algiers, Algeria Univ Sci & Technol Algiers Algeria nol, Neurobiol Lab, Algiers, Algeria INSERM U128, Grp Muscles & Pathol, F-34196 Montpellier, France INSERM U128 Montpellier France F-34196 thol, F-34196 Montpellier, France INSERM EMI 99 18, Lab Physiopathol Cellulaire & Mol Retine, F-67091 Strasbourg, France INSERM EMI 99 18 Strasbourg France F-67091 e, F-67091 Strasbourg, France
Titolo Testata:
BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS
fascicolo: 2, volume: 274, anno: 2000,
pagine: 275 - 280
SICI:
0006-291X(20000802)274:2<275:MEOASD>2.0.ZU;2-7
Fonte:
ISI
Lingua:
ENG
Soggetto:
DUCHENNE MUSCULAR-DYSTROPHY; MDX MOUSE; PROTEINS; UTROPHIN; DP71; GENE; TRANSCRIPT; ISOFORMS; MUTATIONS; PROMOTER;
Keywords:
dystrophin; Dp71; Dp71f-like protein; mdx mouse; mdx(3CV) mouse; mitochondria; microsomes; up-regulation;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Life Sciences
Citazioni:
32
Recensione:
Indirizzi per estratti:
Indirizzo: Martinez-Rojas, D IPN, CINVESTAV, Dept Physiol Biophys & Neurosci, Av IPN2508,Ap 14-740, Mexico City, DF, Mexico IPN Av IPN2508,Ap 14-740 Mexico CityDF Mexico , Mexico
Citazione:
O. Chavez et al., "Mitochondrial expression of a short dystrophin-like product with molecularweight of 71 kDa", BIOC BIOP R, 274(2), 2000, pp. 275-280

Abstract

In the brain, Dp71 is the most abundant protein product of the DMD gene and by alternative splicing of exon 78 two isoforms can be expressed, Dp71d and Dp71f. To explore the subcellular distribution of these Dp71 isoforms, specific monoclonal antibodies were used, Dp71d (with exon 78) was found in microsomes, while Dp71f (without exon 78) was detected in mitochondria. To determine the alterations which the absence of dystrophin proteins induces,we compared the expression of Dp71d in microsomes and Dp71f in mitochondria from mdx and mdx(3CV) mice. Dp71d in microsomes of mdx was similar to that of wild-type mice and, as expected, in mdx(3CV) this protein was undetectable. However, in mitochondria from mdx(3CV), Dp71f was overexpressed in comparison to mitochondria from mdx mice. Because in mdx(3CV) mice all the dystrophin proteins are mutated or diminished, we concluded that the protein detected in mitochondria is not a Dp71f but a novel product named Dp71f-like protein. (C) 2000 Academic Press.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 25/01/20 alle ore 09:20:38