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Titolo:
Defect in N-glycosylation of proteins is tissue-dependent in congenital disorders of glycosylation Ia
Autore:
Dupre, T; Barnier, A; de Lonlay, P; Cormier-Daire, V; Durand, G; Codogno, P; Seta, N;
Indirizzi:
Hop Bichat Claude Bernard, Biochim Lab A, F-75877 Paris 18, France Hop Bichat Claude Bernard Paris France 18 ab A, F-75877 Paris 18, France Hop Necker Enfants Malad, Serv Pediat & Malad Metab, INSERM, U393, F-75743Paris, France Hop Necker Enfants Malad Paris France F-75743 U393, F-75743Paris, France Hop Necker Enfants Malad, Serv Genet Med, INSERM, U393, F-75743 Paris 15, France Hop Necker Enfants Malad Paris France 15 U393, F-75743 Paris 15, France INSERM, U504, F-94807 Villejuif, France INSERM Villejuif France F-94807INSERM, U504, F-94807 Villejuif, France Univ Paris 11, Fac Pharm, F-92260 Chatenay Malabry, France Univ Paris 11 Chatenay Malabry France F-92260 0 Chatenay Malabry, France Univ Paris 05, Fac Pharm, F-75270 Paris 06, France Univ Paris 05 Paris France 06 is 05, Fac Pharm, F-75270 Paris 06, France
Titolo Testata:
GLYCOBIOLOGY
fascicolo: 12, volume: 10, anno: 2000,
pagine: 1277 - 1281
SICI:
0959-6658(200012)10:12<1277:DINOPI>2.0.ZU;2-Y
Fonte:
ISI
Lingua:
ENG
Soggetto:
DEFICIENT-GLYCOPROTEIN-SYNDROME; BETA-TRACE PROTEIN; PHOSPHOMANNOMUTASE DEFICIENCY; LINKED OLIGOSACCHARIDES; CEREBROSPINAL-FLUID; SERUM GLYCOPROTEINS; TRANSFERRIN; EXPRESSION; TRANSPORT; GENE;
Keywords:
alpha 1-antitrypsin; CDG; GLUT-1; immunoglobulin G; protein glycosylation;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Life Sciences
Citazioni:
31
Recensione:
Indirizzi per estratti:
Indirizzo: Dupre, T Hop Bichat Claude Bernard, Biochim Lab A, 46 Rue H Huchard, F-75877 Paris 18, France Hop Bichat Claude Bernard 46 Rue H Huchard Paris France 18 rance
Citazione:
T. Dupre et al., "Defect in N-glycosylation of proteins is tissue-dependent in congenital disorders of glycosylation Ia", GLYCOBIOLOG, 10(12), 2000, pp. 1277-1281

Abstract

The biochemical hallmark of Congenital Disorders of Glycosylation (CDG) including type Ia is a defective N-glycosylation of serum glycoproteins. Hypoglycosylated forms of al-antitrypsin have been detected by Western blot in serum from CDG Ia patients. In contrast we were not able to detect hypoglycosylation in al-antitrypsin synthesized by fibroblasts, keratinocytes, enterocytes, and leukocytes, Similarly no hypoglycosylation was detectable in amembrane-associated N-linked glycoprotein, the facilitative glucose transporter GLUT-1 and also in serum immunoglobulin G isolated from sera of CDG Ia patients, We conclude that the phenotypic expression of CDG Ia is tissue-dependent.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 30/11/20 alle ore 20:14:25