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Titolo:
Alteration of mannose transport in fibroblasts from type I carbohydrate deficient glycoprotein syndrome patients
Autore:
Dupre, T; Ogier-Denis, E; Moore, SEH; Cormier-Daire, V; Dehoux, M; Durand, G; Seta, N; Codogno, P;
Indirizzi:
Hop Bichat, Biochim Lab A, F-75877 Paris 18, France Hop Bichat Paris France 18 chat, Biochim Lab A, F-75877 Paris 18, France Fac Med Xavier Bichat, INSERM, U410, F-75870 Paris, France Fac Med Xavier Bichat Paris France F-75870 , U410, F-75870 Paris, France Hopranceer Enfants Malad, INSERM, U393, Serv Genet Med, F-75743 Paris 15, F Hop Necker Enfants Malad Paris France 15 Genet Med, F-75743 Paris 15, F Univ Paris 11, Fac Pharm, F-92296 Chatenay Malabry, France Univ Paris 11 Chatenay Malabry France F-92296 6 Chatenay Malabry, France Univ Paris 05, Fac Pharm, F-75270 Paris 6, France Univ Paris 05 Paris France 6 aris 05, Fac Pharm, F-75270 Paris 6, France
Titolo Testata:
BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE
fascicolo: 3, volume: 1453, anno: 1999,
pagine: 369 - 377
SICI:
0925-4439(19990330)1453:3<369:AOMTIF>2.0.ZU;2-6
Fonte:
ISI
Lingua:
ENG
Soggetto:
PHOSPHOMANNOMUTASE DEFICIENCY; SERUM TRANSFERRIN;
Keywords:
carbohydrate deficient glycoprotein syndrome; mannose uptake; phosphomannomutase; phosphomannose isomerase;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Life Sciences
Citazioni:
26
Recensione:
Indirizzi per estratti:
Indirizzo: Dupre, T Hop Bichat, Biochim Lab A, F-75877 Paris 18, France Hop Bichat Paris France 18 chim Lab A, F-75877 Paris 18, France
Citazione:
T. Dupre et al., "Alteration of mannose transport in fibroblasts from type I carbohydrate deficient glycoprotein syndrome patients", BBA-MOL BAS, 1453(3), 1999, pp. 369-377

Abstract

The aim of the present study was to explore how mannose enters fibroblastsderived from a panel of children suffering from different subtypes of typeI carbohydrate deficient glycoprotein syndrome: seven carbohydrate deficient glycoprotein syndrome subtype Ia (phosphomannomutase deficiency), two carbohydrate deficient glycoprotein syndrome subtype Ib (phosphomannose isomerase deficiency) and two carbohydrate deficient glycoprotein syndrome subtype Ix (not identified deficiency). We showed that a specific mannose transport system exists in all the cells tested but has different characteristicswith respect to carbohydrate deficient glycoprotein syndrome subtypes, Subtype Ia fibroblasts presented a mannose uptake equivalent or higher (maximum 1.6-fold) than control cells with a D-[2-H-3]-mannose incorporation in nascent N-glycoproteins decreased up to 7-fold. Compared to control cells, the mannose uptake was greatly stimulated in subtype Ib (4.0-fold), due to lower K-uptake and higher V-max values. Subtype Ib cells showed an increased incorporation of D-[2-H-3]mannose into nascent N-glycoproteins, Subtype Ix fibroblasts presented an intermediary status with mannose uptake equivalentto the control but with an increased incorporation of D-[2-H-3]-mannose innascent N-glycoproteins. All together, our results demonstrate quantitative and/or qualitative modifications in mannose transport of all carbohydratedeficient glycoprotein syndrome fibroblasts in comparison to control cells, with a relative homogeneity within a considered subtype of carbohydrate deficient glycoprotein syndrome. These results are consistent with the possible use of mannose as a therapeutic agent in carbohydrate deficient glycoprotein syndrome Ib and Ix. (C) 1999 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 30/11/20 alle ore 06:49:58