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Titolo:
beta-hexosaminidase, alpha-D-mannosidase, and beta-mannosidase expression in serum from patients with carbohydrate-deficient glycoprotein syndrome type I
Autore:
Beccari, T; Mancuso, F; Costanzi, E; Tassi, C; Barone, R; Fiumara, A; Orlacchio, A; Aisa, MC; Orlacchio, A;
Indirizzi:
Univ Perugia, Dipartimento Sci Biochim & Biotecnol Mol, Sez Biochim & BiolMol, I-06126 Perugia, Italy Univ Perugia Perugia Italy I-06126 him & BiolMol, I-06126 Perugia, Italy Univ Catania, Pediat Clin, Catania, Italy Univ Catania Catania ItalyUniv Catania, Pediat Clin, Catania, Italy Univ Toronto, Ctr Res Neurodegenerat Dis, Toronto, ON, Canada Univ Toronto Toronto ON Canada s Neurodegenerat Dis, Toronto, ON, Canada
Titolo Testata:
CLINICA CHIMICA ACTA
fascicolo: 1-2, volume: 302, anno: 2000,
pagine: 125 - 132
SICI:
0009-8981(200012)302:1-2<125:BAABEI>2.0.ZU;2-H
Fonte:
ISI
Lingua:
ENG
Soggetto:
OLIGOSACCHARIDE; 6-SULFATE;
Keywords:
CDG syndrome; hexosaminidase; alpha-D-mannosidase; beta-D-mannosidase;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Life Sciences
Citazioni:
19
Recensione:
Indirizzi per estratti:
Indirizzo: Beccari, T Univ Perugia, Dipartimento Sci Biochim & Biotecnol Mol, Sez Biochim & BiolMol, Via del Giochetto, I-06126 Perugia, Italy Univ Perugia Via del Giochetto Perugia Italy I-06126 ia, Italy
Citazione:
T. Beccari et al., "beta-hexosaminidase, alpha-D-mannosidase, and beta-mannosidase expression in serum from patients with carbohydrate-deficient glycoprotein syndrome type I", CLIN CHIM A, 302(1-2), 2000, pp. 125-132

Abstract

The activity of beta -hexosaminidase, determined \Nith 4-methylumbelliferyl-beta -N-acetylglucopyranoside substrate, and of beta -D-mannosidase was significantly higher in the serum of patients with carbohydrate-deficient glycoprotein (CDG) syndrome type IA (phosphomannomutase deficiency) than in controls. No significant differences were observed in the activity of beta -hexosaminidase, determined using 4-methylumbelliferyl-beta -N-acetylglucopyranoside-6-sulphate as substrate, and the activity of alpha -D-mannosidase. Using DEAE-cellulose chromatography, a greater amount of hexosaminidase B than hexosaminidase A was detected in CDG serum. In CDG serum, hexosaminidase A was eluted in a more basic position in the salt gradient. An isoenzymeof alpha -D-mannosidase and beta -D-mannosidase was identified in control and CDG sera. alpha -D-Mannosidase isoenzyme was eluted in a slightly more basic position in CDG serum than in control serum, whereas beta -D-mannosidase isoenzyme was eluted in the same position. (C) 2000 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 21/09/20 alle ore 09:46:09