Multiple sulfatase deficiency: catalytically inactive sulfatases are expressed from retrovirally introduced sulfatase cDNAs
about
Ethanol decreases rat hepatic arylsulfatase A activity levelsExpression, localization, structural, and functional characterization of pFGE, the paralog of the Calpha-formylglycine-generating enzyme.Conversion of cysteine to formylglycine: a protein modification in the endoplasmic reticulum.Rapid degradation of an active formylglycine generating enzyme variant leads to a late infantile severe form of multiple sulfatase deficiency.Formylglycine, a post-translationally generated residue with unique catalytic capabilities and biotechnology applications.Heparan N-sulfatase: cysteine 70 plays a role in the enzyme catalysis and processing.The non-catalytic N-terminal extension of formylglycine-generating enzyme is required for its biological activity and retention in the endoplasmic reticulum.Overexpression of inactive arylsulphatase mutants and in vitro activation by light-dependent oxidation with vanadate.Conversion of cysteine to formylglycine in eukaryotic sulfatases occurs by a common mechanism in the endoplasmic reticulum.Characterization of iduronate sulphatase mutants affecting N-glycosylation sites and the cysteine-84 residueImmortalization and characterization of a cell line exhibiting a severe multiple sulphatase deficiency phenotype.Overexpression of N-acetylgalactosamine-4-sulphatase induces a multiple sulphatase deficiency in mucopolysaccharidosis-type-VI fibroblasts.SUMF1 mutations affecting stability and activity of formylglycine generating enzyme predict clinical outcome in multiple sulfatase deficiency.First evidences for a third sulfatase maturation system in prokaryotes from E. coli aslB and ydeM deletion mutants.
P2860
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P2860
Multiple sulfatase deficiency: catalytically inactive sulfatases are expressed from retrovirally introduced sulfatase cDNAs
description
1992 nî lūn-bûn
@nan
1992年の論文
@ja
1992年論文
@yue
1992年論文
@zh-hant
1992年論文
@zh-hk
1992年論文
@zh-mo
1992年論文
@zh-tw
1992年论文
@wuu
1992年论文
@zh
1992年论文
@zh-cn
name
Multiple sulfatase deficiency: ...... lly introduced sulfatase cDNAs
@en
type
label
Multiple sulfatase deficiency: ...... lly introduced sulfatase cDNAs
@en
prefLabel
Multiple sulfatase deficiency: ...... lly introduced sulfatase cDNAs
@en
P2860
P356
P1476
Multiple sulfatase deficiency: ...... lly introduced sulfatase cDNAs
@en
P2093
Rommerskirch W
von Figura K
P2860
P304
P356
10.1073/PNAS.89.7.2561
P407
P577
1992-04-01T00:00:00Z