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Rapid degradation of an active formylglycine generating enzyme variant leads to a late infantile severe form of multiple sulfatase deficiency

Multiple sulfatase deficiency (MSD) is a rare inborn error of metabolism affecting posttranslational activation of sulfatases by the formylglycine generating enzyme (FGE). Due to mutations in the encoding SUMF1 gene, FGE's catalytic capacity is impaired resulting in reduced cellular sulfatase a...

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Autori principali: Schlotawa, Lars, Radhakrishnan, Karthikeyan, Baumgartner, Matthias, Schmid, Regula, Schmidt, Bernhard, Dierks, Thomas, Gärtner, Jutta
Natura: Artigo
Lingua:Inglês
Pubblicazione: Nature Publishing Group 2013
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Accesso online:https://ncbi.nlm.nih.gov/pmc/articles/PMC3746267/
https://ncbi.nlm.nih.gov/pubmed/23321616
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1038/ejhg.2012.291
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