Sulfatase-modifying factor: Difference between revisions
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Mutations in SUMF1 cause multiple sulfatase deficiency - a lysosomal storage disorder. SUMF1 is associated with chronic obstructive pulmonary disease<ref>PMID:28464818</ref>. | Mutations in SUMF1 cause multiple sulfatase deficiency - a lysosomal storage disorder. SUMF1 is associated with chronic obstructive pulmonary disease<ref>PMID:28464818</ref>. | ||
== | == Structural highlights == | ||
The structure of SUMF1 complex with its substrate sulfatase terminal peptide CTPSR. SUMF1 active site contains 2 cysteine residues and mutating either of them to serine results in an inactive enzyme. Cys341 was found to be responsible for substrate binding and makes a Cys-Cys bond to the peptide cysteine residue. The peptide binds at the surface of SUMF1 in an extended conformation making numerous interactions with the protein<ref>PMID:16368756</ref>. | |||
</StructureSection> | </StructureSection> | ||
