Inhibition of iduronic acid biosynthesis by ebselen reduces glycosaminoglycan accumulation in mucopolysaccharidosis type I fibroblasts.

Maccarana, Marco; Tykesson, Emil; Pera, Edgar M; Gouignard, Nadège; Fang, Jianping; Malmström, Anders; Ghiselli, Giancarlo; Li, Jin-Ping

Maccarana, Marco; Tykesson, Emil; Pera, Edgar M; Gouignard, Nadège; Fang, Jianping; Malmström, Anders; Ghiselli, Giancarlo; Li, Jin-Ping.

Affiliation

Maccarana M; Department of Medical Biochemistry and Microbiology, BMC B11, Uppsala University, Husargatan 3 Box 582 751 23 Uppsala, Sweden.
Tykesson E; Department of Experimental Medical Science, BMC C12, Lund University, BMC H11, 221 84 Lund, Sweden.
Pera EM; Department of Experimental Medical Science, BMC C12, Lund University, BMC H11, 221 84 Lund, Sweden.
Gouignard N; Department of Laboratory Medicine, Lund Stem Cell Center, Lund University, BMC H11, 221 84 Lund, Sweden.
Fang J; Department of Laboratory Medicine, Lund Stem Cell Center, Lund University, BMC H11, 221 84 Lund, Sweden.
Malmström A; GlycoNovo Technologies Co., Ltd., Shanghai 201203, China.
Ghiselli G; Department of Experimental Medical Science, BMC C12, Lund University, BMC H11, 221 84 Lund, Sweden.
Li JP; Glyconova Srl, Parco Scientifico Silvano Fumero, Bioindustry Park Silvano Fumero S.p.A Via Ribes, 5 - 10010 - Colleretto Giacosa (TO), Italy.

Glycobiology ; 31(10): 1319-1329, 2021 11 18.

Article de En | MEDLINE | ID: mdl-34192316

Sujet(s)
Mots clés

chondroitin dermatan sulfate; ebselen; epimerases; mucopolysaccharidosis type I; substrate reduction therapy

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Texte intégral: 1 Collection: 01-internacional Base de données: MEDLINE Sujet principal: Composés organiques du sélénium / Mucopolysaccharidose de type I / Isoindoles / Fibroblastes / Glycosaminoglycanes / Acide iduronique Limites: Humans Langue: En Journal: Glycobiology Sujet du journal: BIOQUIMICA Année: 2021 Type de document: Article Pays d'affiliation: Suède

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