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A reference for what the literature reports. Not medical advice.

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Proteins/UNIPROT:Q8NBK3

SUMF1

Protein·UNIPROT:Q8NBK3Formylglycine-generating enzyme

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Oxidase that catalyzes the conversion of cysteine to 3-oxoalanine on target proteins, using molecular oxygen and an unidentified reducing agent. 3-oxoalanine modification, which is also named formylglycine (fGly), occurs in the maturation of arylsulfatases and some alkaline phosphatases that use the hydrated form of 3-oxoalanine as a catalytic nucleophile. Known substrates include GALNS, ARSA, STS and ARSE..

Function from UniProtKB (Q8NBK3), read 2026-09-23. CC BY 4.0.

Curated compounds routed: 0

What the reference databases state

Inferred

Inferred — not reported by a source. Evidence key

GenCC submitters' classifications

1 row

mucosulfatidosis has a germline basis in SUMF1DiseaseGenCC HGNC:20376|MONDO:0010088Inferred only

Statements

16 statements

16 statements · 4 reference databases · Subject: SUMF1 · Sources: Bgee, Reactome, Rhea, UniProt

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SUMF1 is expressed in layer of synovial tissueAnatomyBgee ENSG00000144455Inferred only
SUMF1 is expressed in synovial jointAnatomyBgee ENSG00000144455Inferred only
SUMF1 is expressed in upper arm skinAnatomyBgee ENSG00000144455Inferred only
SUMF1 participates in Gamma carboxylation, hypusinylation, hydroxylation, and arylsulfatase activationPathwayReactome R-HSA-163841Inferred only
SUMF1 participates in Glycosphingolipid catabolismPathwayReactome R-HSA-9840310Inferred only
SUMF1 participates in Glycosphingolipid metabolismPathwayReactome R-HSA-1660662Inferred only
SUMF1 participates in MetabolismPathwayReactome R-HSA-1430728Inferred only
SUMF1 participates in Metabolism of lipidsPathwayReactome R-HSA-556833Inferred only
SUMF1 participates in Metabolism of proteinsPathwayReactome R-HSA-392499Inferred only
SUMF1 participates in Post-translational protein modificationPathwayReactome R-HSA-597592Inferred only
SUMF1 participates in Sphingolipid metabolismPathwayReactome R-HSA-428157Inferred only
SUMF1 participates in The activation of arylsulfatasesPathwayReactome R-HSA-1663150Inferred only

Show the remaining 4

SUMF1 requires the cofactor Cu(2+)CompoundUniProt Q8NBK3Inferred only
a thiol is a substrate of SUMF1CompoundRhea RHEA:51152Inferred only
an organic disulfide is a substrate of SUMF1CompoundRhea RHEA:51152Inferred only
hydrogen sulfide is a substrate of SUMF1CompoundRhea RHEA:51152Inferred only

Reactions

1 reaction

L-cysteinyl-[sulfatase]+2a thiol+dioxygenan organic disulfide+3-oxo-L-alanyl-[sulfatase]+hydrogen sulfide+water+hydron
Catalysed by SUMF1
Reaction pageDirection not assertedBalance undeterminable
Rhea RHEA:51152CC BY 4.0Reference data

Other names

3 names

Resolves to: SUMF1

Also called: C-alpha-formylglycine-generating enzyme 1FGESulfatase-modifying factor 1

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