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Titolo:
IMMUNOCYTOCHEMISTRY OF MUSCLE CYTOSKELETAL PROTEINS IN ACID MALTASE DEFICIENCY
Autore:
VITA G; MIGLIORATO A; TOSCANO A; BORDONI A; BRESOLIN N; FIUMARA A; MESSINA C;
Indirizzi:
UNIV MESSINA POLICLIN,NEUROL CLIN 2 I-98125 MESSINA ITALY UNIV MESSINA,INST NEUROL & NEUROSURG SCI MESSINA ITALY UNIV MILAN,DINO FERRARI CTR,INST CLIN NEUROL MILAN ITALY UNIV CATANIA,INST PEDIAT CATANIA ITALY
Titolo Testata:
Muscle & nerve
fascicolo: 6, volume: 17, anno: 1994,
pagine: 655 - 661
SICI:
0148-639X(1994)17:6<655:IOMCPI>2.0.ZU;2-5
Fonte:
ISI
Lingua:
ENG
Soggetto:
DUCHENNE MUSCULAR-DYSTROPHY; GLYCOGENOSIS TYPE-II; SKELETAL-MUSCLE; INTERMEDIATE FILAMENTS; LOCALIZATION; VINCULIN; MYOPATHY; DESMIN; JUNCTIONS; VIMENTIN;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Science Citation Index Expanded
Citazioni:
34
Recensione:
Indirizzi per estratti:
Citazione:
G. Vita et al., "IMMUNOCYTOCHEMISTRY OF MUSCLE CYTOSKELETAL PROTEINS IN ACID MALTASE DEFICIENCY", Muscle & nerve, 17(6), 1994, pp. 655-661

Abstract

Immunocytochemistry of dystrophin, spectrin, vinculin (sarcolemma-specific proteins), and desmin (an intermediate filament protein) were investigated in 5 patients with acid maltase deficiency (AMD). One patient had infant onset, 2 had childhood onset, and 2 had adult onset. Allhad a vacuolar myopathy with autophagic vacuoles containing glycogen and cytoplasmic degradation products. Dystrophin, spectrin, and vinculin were localized at the sarcolemma as in normal muscle fibers. Withinthe cytoplasm of many fibers, immunoreactivity for the three proteinswas seen as single or multiple spots or as circular structures, whichmost likely corresponded to the limiting membrane of vacuoles. Desminwas overexpressed at the periphery of some vacuoles. It is plausible that, before exocytosis occurs, sarcolemma-specific proteins appear within the vacuole membrane. Vacuole immunolabeling frequently occurred in the patients with childhood and adult onset AMD, but very rarely occurred in the case with infant onset. We hypothesize that a reduced exocytosis rate might explain the infrequent vacuole immunolabeling and the early onset of the infant form of the disease. (C) 1994 John Wiley& Sons, Inc.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 27/11/20 alle ore 14:47:06