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beta-hexosaminidase, alpha-D-mannosidase, and beta-mannosidase expression in serum from patients with carbohydrate-deficient glycoprotein syndrome type I.
Beccari, T; Mancuso, F; Costanzi, E; Tassi, C; Barone, R; Fiumara, A; Orlacchio, A; Aisa, M C; Orlacchio, A.
Afiliação
  • Beccari T; Dipartimento di Scienze Biochimiche e Biotecnologie Molecolari, Sezione di Biochimica e Biologia Molecolare, Università degli Studi di Perugia, Via del Giochetto, 06126, Perugia, Italy. tbeccari@unipg.it
Clin Chim Acta ; 302(1-2): 125-32, 2000 Dec.
Article em En | MEDLINE | ID: mdl-11074069
The activity of beta-hexosaminidase, determined with 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 isoenzyme of 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.
Assuntos
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Base de dados: MEDLINE Assunto principal: Beta-N-Acetil-Hexosaminidases / Defeitos Congênitos da Glicosilação / Manosidases Limite: Adolescent / Adult / Female / Humans / Male Idioma: En Ano de publicação: 2000 Tipo de documento: Article
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Base de dados: MEDLINE Assunto principal: Beta-N-Acetil-Hexosaminidases / Defeitos Congênitos da Glicosilação / Manosidases Limite: Adolescent / Adult / Female / Humans / Male Idioma: En Ano de publicação: 2000 Tipo de documento: Article