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Titolo:
Early and selective disappearance of telethonin protein from the sarcomerein neurogenic atrophy
Autore:
Schroder, R; Reimann, J; Iakovenko, A; Mues, A; Bonnemann, CG; Matten, J; Gautel, M;
Indirizzi:
Univ Bonn, Dept Neurol, D-53127 Bonn, Germany Univ Bonn Bonn Germany D-53127 Bonn, Dept Neurol, D-53127 Bonn, Germany Univ Gottingen, Dept Pediat, D-37075 Gottingen, Germany Univ Gottingen Gottingen Germany D-37075 iat, D-37075 Gottingen, Germany Max Planck Inst Mol Physiol, D-44227 Dortmund, Germany Max Planck Inst MolPhysiol Dortmund Germany D-44227 7 Dortmund, Germany
Titolo Testata:
JOURNAL OF MUSCLE RESEARCH AND CELL MOTILITY
fascicolo: 3, volume: 22, anno: 2001,
pagine: 259 - 264
SICI:
0142-4319(2001)22:3<259:EASDOT>2.0.ZU;2-K
Fonte:
ISI
Lingua:
ENG
Soggetto:
MUSCLE-SPECIFIC CALPAIN; HUMAN SKELETAL-MUSCLE; MONOCLONAL-ANTIBODIES; TITIN; FILAMENTS; P94;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Life Sciences
Citazioni:
18
Recensione:
Indirizzi per estratti:
Indirizzo: Schroder, R Univ Bonn, Dept Neurol, Sigmund Freud Str 25, D-53127 Bonn, Germany Univ Bonn Sigmund Freud Str 25 Bonn Germany D-53127 , Germany
Citazione:
R. Schroder et al., "Early and selective disappearance of telethonin protein from the sarcomerein neurogenic atrophy", J MUSCLE R, 22(3), 2001, pp. 259-264

Abstract

Mutations of the human telethonin gene have recently been shown to cause limb girdle muscular dystrophy type 2G in three Brazilian families. The mRNAhas been shown to be dynamically regulated in animals, however, the fate of the protein in human muscle is unknown. In order to assess the expressionof telethonin in more frequently encountered myopathological conditions wegenerated and characterized a rabbit antiserum raised against the C-terminal end of telethonin by immunoblotting and immunogold EM. Indirect immunofluorescence analysis of a wide variety of neuromuscular disorders including dystrophinopathies, metabolic myopathies, denervation disorders, congenitaland inflammatory myopathies revealed that the characteristic Z-band staining of telethonin was preserved in all disease entities included in our study. However, a reduced telethonin immunoreactivity was observed in up to 10%of type II fibers in 10 cases of neurogenic atrophy. A decreased telethonin staining was more frequently observed in early stages of fiber atrophy than in type II fibers displaying normal or highly atrophic fiber diameters. Hence, not only the telethonin transcript is rapidly downregulated in denervated muscle but the protein itself undergoes dynamic changes while its known sarcomeric binding partner titin remains unaltered. Beyond its role as astatic component of Z-bands, these findings indicate that telethonin protein levels seems to be at least in part regulated by neuronal activity and is thus linked to the dynamic control of myofibrillogenesis and muscle turnover in human skeletal muscle.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 26/11/20 alle ore 20:28:18