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Titolo:
Congenital disorders of glycosylation: genetic model systems lead the way
Autore:
Aebi, M; Hennet, T;
Indirizzi:
ETH Zurich, Inst Mikrobiol, CH-8092 Zurich, Switzerland ETH Zurich Zurich Switzerland CH-8092 obiol, CH-8092 Zurich, Switzerland Univ Zurich, Inst Physiol, CH-8006 Zurich, Switzerland Univ Zurich Zurich Switzerland CH-8006 siol, CH-8006 Zurich, Switzerland
Titolo Testata:
TRENDS IN CELL BIOLOGY
fascicolo: 3, volume: 11, anno: 2001,
pagine: 136 - 141
SICI:
0962-8924(200103)11:3<136:CDOGGM>2.0.ZU;2-0
Fonte:
ISI
Lingua:
ENG
Soggetto:
DEFICIENT GLYCOPROTEIN SYNDROME; PHOSPHOMANNOSE ISOMERASE DEFICIENCY; N-LINKED OLIGOSACCHARIDES; HAMSTER OVARY CELLS; PROTEIN GLYCOSYLATION; I DEFICIENCY; CDG SYNDROME; MUTATIONS; BIOSYNTHESIS; BIOCHEMISTRY;
Tipo documento:
Review
Natura:
Periodico
Settore Disciplinare:
Life Sciences
Citazioni:
47
Recensione:
Indirizzi per estratti:
Indirizzo: Aebi, M ETH Zurich, Inst Mikrobiol, Schmelzbergstr 7, CH-8092 Zurich, Switzerland ETH Zurich Schmelzbergstr 7 Zurich Switzerland CH-8092 witzerland
Citazione:
M. Aebi e T. Hennet, "Congenital disorders of glycosylation: genetic model systems lead the way", TR CELL BIO, 11(3), 2001, pp. 136-141

Abstract

N-linked glycosylation is the most frequent modification of secretory proteins in eukaryotic cells. The highly conserved glycosylation process is initiated in the endoplasmic reticulum (ER), where the Glc(3)Man(9)GlcNAc(2) oligosaccharide is assembled on the lipid carrier dolichylpyrophosphate and then transferred to selected asparagine residues of polypeptide chains. In recent years, several inherited human diseases, congenital disorders of glycosylation (CDG), have been associated with deficiencies in this pathway. The ER-associated glycosylation pathway has been studied in the budding yeast Saccharomyces cerevisiae, and this model system has been invaluable in elucidating the molecular basis of novel types of CDG.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 19/09/20 alle ore 12:43:05