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| <StructureSection load='2aij' size='340' side='right' caption='Caption for this structure' scene=''> | | <StructureSection load='2aij' size='340' side='right' caption='Glycosylated human sulfatase-modifying factor 1 (grey) complex with arylsulfatase peptide (green), Ca+2 ion (green) and Cl- ion (green) (PDB code [[2aij]]) ' scene=''> |
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| == Function == | | == Function == |
Revision as of 10:02, 11 November 2018
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Function
Sulfatase-modifying factor (SUMF) is an enzyme that catalyzes the hydrolysis of sulfate esters by oxidizing posttranslationally a cysteine residue in its substrate Sulfatase active site to 3-oxoalanine (C-α-formylglycine) residue[1].
Disease
Mutations in SUMF1 cause multiple sulfatase deficiency - a lysosomal storage disorder. SUMF1 is associated with chronic obstructive pulmonary disease[2].
Relevance
Structural highlights
- ↑ Zito E, Buono M, Pepe S, Settembre C, Annunziata I, Surace EM, Dierks T, Monti M, Cozzolino M, Pucci P, Ballabio A, Cosma MP. Sulfatase modifying factor 1 trafficking through the cells: from endoplasmic reticulum to the endoplasmic reticulum. EMBO J. 2007 May 16;26(10):2443-53. doi: 10.1038/sj.emboj.7601695. Epub 2007 Apr , 19. PMID:17446859 doi:https://dx.doi.org/10.1038/sj.emboj.7601695
- ↑ Weidner J, Jarenback L, de Jong K, Vonk JM, van den Berge M, Brandsma CA, Boezen HM, Sin D, Bosse Y, Nickle D, Ankerst J, Bjermer L, Postma DS, Faiz A, Tufvesson E. Sulfatase modifying factor 1 (SUMF1) is associated with Chronic Obstructive Pulmonary Disease. Respir Res. 2017 May 2;18(1):77. doi: 10.1186/s12931-017-0562-5. PMID:28464818 doi:https://dx.doi.org/10.1186/s12931-017-0562-5
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3D structures of sulfatase-modifying factor
Updated on 11-November-2018
Sulfatase - hSUMF-1 - human
2aft, 2afy, 2hib, 2hi8 - hSUMF-1 (mutant)
2aij, 2aik - hSUMF-1 (mutant) + peptide
1y4j - hSUMF-2
References
