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Titolo:
Elevated beta-N-acetylhexosaminidase activity in focal dystonia fibroblasts
Autore:
Costanzi, E; Beccari, T; Tassi, C; Orlacchio, A; Rossi, A;
Indirizzi:
Univ Perugia, Dipartimento Med Sperimentale & Sci Biochim, I-06126 Perugia, Italy Univ Perugia Perugia Italy I-06126 & Sci Biochim, I-06126 Perugia, Italy Univ Perugia, Dipartimento Biol Cellulare & Mol, I-06100 Perugia, Italy Univ Perugia Perugia Italy I-06100 llulare & Mol, I-06100 Perugia, Italy Univ Perugia, Ist Neurol, I-06126 Perugia, Italy Univ Perugia Perugia Italy I-06126 a, Ist Neurol, I-06126 Perugia, Italy
Titolo Testata:
CLINICA CHIMICA ACTA
fascicolo: 1-2, volume: 288, anno: 1999,
pagine: 31 - 35
SICI:
0009-8981(199910)288:1-2<31:EBAIFD>2.0.ZU;2-N
Fonte:
ISI
Lingua:
ENG
Soggetto:
DISORDERS;
Keywords:
dystonia; lysosomal enzymes; torticollis; writer's cramp;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Life Sciences
Citazioni:
13
Recensione:
Indirizzi per estratti:
Indirizzo: Tassi, C Univ Perugia, Dipartimento Med Sperimentale & Sci Biochim, Via Giochetto, I-06126 Perugia, Italy Univ Perugia Via Giochetto Perugia Italy I-06126 Perugia, Italy
Citazione:
E. Costanzi et al., "Elevated beta-N-acetylhexosaminidase activity in focal dystonia fibroblasts", CLIN CHIM A, 288(1-2), 1999, pp. 31-35

Abstract

Specific activities of beta-D-hexosaminidase, alpha-D-mannosidase, beta-D-galactosidase and beta-D-glucuronidase were determined in fibroblasts of patients with writer's cramp and torticollis. These diseases show degenerative neurological disorders similar to those observed in lysosomal diseases. Hexosaminidase specific activities, determined using 4-methylumbelliferyl-beta-N-acetylglucopyranoside and 4-methylumbelliferyl-beta-N-acetylglucopyranoside-6-sulphate as substrates, were significantly higher in the fibroblasts of patients than in controls. No significant differences were observed inthe specific activities of the other lysosomal enzymes. The increased hexosaminidase specific activities in torticollis and writer's cramp may be additional markers for these diseases. (C) 1999 Elsevier Science B.V. All rights reserved.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 28/09/20 alle ore 14:53:10