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Titolo:
ABNORMAL EXPRESSION OF INTERMEDIATE FILAMENT PROTEINS IN X-LINKED MYOTUBULAR MYOPATHY IS NOT REPRODUCED IN-VITRO
Autore:
VANDERVEN PFM; JAP PHK; BARTH PG; SENGERS RCA; RAMAEKERS FCS; STADHOUDERS AM;
Indirizzi:
UNIV NIJMEGEN,DEPT CELL BIOL & HISTOL,TRIGON BLDG,POB 9101 6500 HB NIJMEGEN NETHERLANDS UNIV AMSTERDAM,ACAD MED CTR,DEPT PEDIAT 1105 AZ AMSTERDAM NETHERLANDS UNIV AMSTERDAM,ACAD MED CTR,DEPT NEUROL 1105 AZ AMSTERDAM NETHERLANDS UNIV NIJMEGEN HOSP,DEPT PEDIAT 6500 HB NIJMEGEN NETHERLANDS UNIV LIMBURG,DEPT MOLEC CELL BIOL & GENET 6200 MD MAASTRICHT NETHERLANDS
Titolo Testata:
Neuromuscular disorders
fascicolo: 4, volume: 5, anno: 1995,
pagine: 267 - 275
SICI:
0960-8966(1995)5:4<267:AEOIFP>2.0.ZU;2-E
Fonte:
ISI
Lingua:
ENG
Soggetto:
SKELETAL-MUSCLE CELLS; CENTRONUCLEAR MYOPATHY; MONOCLONAL-ANTIBODY; CENTRAL NUCLEI; DESMIN; MATURATION; VIMENTIN; MYOGENESIS; CULTURE; LINKAGE;
Keywords:
X-LINKED MYOTUBULAR MYOPATHY; SKELETAL MUSCLE CELL CULTURES; MUSCLE FIBER IMMATURITY; INTERMEDIATE FILAMENT PROTEINS; TITIN; IMMUNOHISTOCHEMISTRY;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Science Citation Index Expanded
Science Citation Index Expanded
Citazioni:
39
Recensione:
Indirizzi per estratti:
Citazione:
P.F.M. Vanderven et al., "ABNORMAL EXPRESSION OF INTERMEDIATE FILAMENT PROTEINS IN X-LINKED MYOTUBULAR MYOPATHY IS NOT REPRODUCED IN-VITRO", Neuromuscular disorders, 5(4), 1995, pp. 267-275

Abstract

Expression patterns of the intermediate filament proteins (IFPs) desmin and vimentin, in biopsy material taken from a 1 day old boy with fatal neonatal X-linked myotubular myopathy (XLMTM) were compared with the expression of these proteins in cultured myotubes, from the same patient. Immunohistochemical studies revealed the persistence of high levels of desmin in virtually all, and vimentin in most, of the myofibres within the patient's biopsy. Analysis of intermediate filament expression in differentiating cultured muscle cells did not reveal overt differences between XLMTM cultures and cultures of control muscle. Titindistribution patterns indicated a normal process of myofibrillogenesis in XLMTM myotubes. We conclude that the failure to properly regulateIFP-expression is not intrinsic to XLMTM muscle fibres. The possibility that this failure is due to a defective external, possibly neural factor, is discussed.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 28/09/20 alle ore 21:47:06