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Titolo:
MICROHETEROGENEITY OF SERUM GLYCOPROTEINS AND THEIR LIVER PRECURSORS IN PATIENTS WITH CARBOHYDRATE-DEFICIENT GLYCOPROTEIN SYNDROME TYPE-I -APPARENT DEFICIENCIES IN CLUSTERIN AND SERUM AMYLOID-P
Autore:
HENRY H; TISSOT JD; MESSERLI B; MARKERT M; MUNTAU A; SKLADAL D; SPERL W; JAEKEN J; WEIDINGER S; HEYNE K; BACHMANN C;
Indirizzi:
CHU VAUDOIS,CENT LAB CLIN CHEM CH-1011 LAUSANNE SWITZERLAND CHU VAUDOIS,CENT LAB CLIN CHEM CH-1011 LAUSANNE SWITZERLAND RED CROSS TRANSFUS CTR LAUSANNE SWITZERLAND UNIV HOSP,DEPT PEDIAT MUNICH GERMANY INNSBRUCK UNIV,DEPT PEDIAT A-6020 INNSBRUCK AUSTRIA UNIV HOSP GASTHUISBERG,DEPT PEDIAT B-3000 LOUVAIN BELGIUM BAVARIAN RED CROSS REGENSBURG GERMANY KINDERARTZ KIEL GERMANY
Titolo Testata:
The Journal of laboratory and clinical medicine
fascicolo: 4, volume: 129, anno: 1997,
pagine: 412 - 421
SICI:
0022-2143(1997)129:4<412:MOSGAT>2.0.ZU;2-W
Fonte:
ISI
Lingua:
ENG
Soggetto:
PROTEINS; TRANSFERRIN; ELECTROPHORESIS; DEGRADATION; DISORDERS; COMPONENT; PLASMA; GOLGI;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Science Citation Index Expanded
Citazioni:
34
Recensione:
Indirizzi per estratti:
Citazione:
H. Henry et al., "MICROHETEROGENEITY OF SERUM GLYCOPROTEINS AND THEIR LIVER PRECURSORS IN PATIENTS WITH CARBOHYDRATE-DEFICIENT GLYCOPROTEIN SYNDROME TYPE-I -APPARENT DEFICIENCIES IN CLUSTERIN AND SERUM AMYLOID-P", The Journal of laboratory and clinical medicine, 129(4), 1997, pp. 412-421

Abstract

Serum and liver protein patterns were studied, respectively, in 5 patients (serum) and 1 patient (liver) with carbohydrate-deficient glycoprotein syndrome (CDGS) type I by high-resolution two-dimensional electrophoresis (2-DE) and sodium dodecyl sulfate-polyacrylamide gel electrophoresis (SDS-PAGE). The pattern of serum glycoproteins in all 5 patients presented abnormal trains of isoforms with decreased mass (delta molecular weight 3000) and all showed a cathodal shift. Two-dimensional electrophoresis and SDS-PAGE mass analysis of transferrin, alpha 1-antitrypsin, haptoglobin beta-chain, and alpha 1-acid glycoprotein after neuraminidase and N-glycosidase F treatments demonstrated that the additional trains of the isoforms found in CDGS type I contain homologous species of Isoforms. Some of them still showed charge differences, and all still contained glycans except for transferrin, with some unusual nonglycosylated isoforms, in addition, deficiencies in clusterin and serum amyloid P, not described so far, have been found in all 5 patients. The two-dimensional pattern of immunodetected precursors of serum proteins in liver cells from ii patient with CDGS showed abnormal low-mass precursors and the absence of the precursors normally found incontrols. These results suggest that these abnormal precursors accumulate during the early oligosaccharide processing of the nascent protein-bound oligosaccharides and that glycoprotein precursors undergo an altered intracellular transport while the post-translational processingalong the normal pathway is still apparently functioning in patients with CDGS.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 09/07/20 alle ore 22:13:42