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Article

Recognition and ER Quality Control of Misfolded Formylglycine-Generating Enzyme by Protein Disulfide Isomerase.

Cell reports - 3 Jul 2018

Schlotawa Lars, Wachs Michaela, Bernhard Olaf, Mayer Franz J, Dierks Thomas, Schmidt Bernhard, Radhakrishnan Karthikeyan

Abstract excerpt

Multiple sulfatase deficiency (MSD) is a fatal, inherited lysosomal storage disorder characterized by reduced activities of all sulfatases in patients. Sulfatases require a unique post-translational modification of an active-site cysteine to formylglycine that is catalyzed by the formylglycine-generating enzyme (FGE). FGE mutations that affect intracellular protein stability determine residual enzyme activity and...

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Identifiers

PMID: 29972788
DOI: 10.1016/j.celrep.2018.06.016

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