InnateDB: Systems Biology of the Innate Immune Response

Homo sapiens Protein: MOCOS

Summary

InnateDB Protein

IDBP-2603.5

Last Modified

2014-10-13 [Report errors or provide feedback]

Gene Symbol

MOCOS

Protein Name

molybdenum cofactor sulfurase

Synonyms

Species

Homo sapiens

Ensembl Protein

ENSP00000261326

InnateDB Gene

IDBG-2601 (MOCOS)

Protein Structure

UniProt Annotation

Function

Sulfurates the molybdenum cofactor. Sulfation of molybdenum is essential for xanthine dehydrogenase (XDH) and aldehyde oxidase (ADO) enzymes in which molybdenum cofactor is liganded by 1 oxygen and 1 sulfur atom in active form. In vitro, the C-terminal domain is able to reduce N-hydroxylated prodrugs, such as benzamidoxime. {ECO:0000255HAMAP-Rule:MF_03050, ECO:0000269PubMed:16973608}.

Subcellular Localization

Disease Associations

Xanthinuria 2 (XU2) [MIM:603592]: A disorder characterized by excretion of very large amounts of xanthine in the urine and a tendency to form xanthine stones. Uric acid is strikingly diminished in serum and urine. In addition, patients suffering of xanthinuria 2 cannot metabolize allopurinol into oxypurinol due to dual deficiency of xanthine dehydrogenase and aldehyde oxidase. {ECO:0000269PubMed:11302742, ECO:0000269PubMed:14624414, ECO:0000269PubMed:17368066, ECO:0000269Ref.10}. Note=The disease is caused by mutations affecting the gene represented in this entry.

Tissue Specificity

Comments

Interactions

Number of Interactions

This gene and/or its encoded proteins are associated with 4 experimentally validated interaction(s) in this database.

Experimentally validated
Total	4 [view]
Protein-Protein	3 [view]
Protein-DNA	1 [view]
Protein-RNA	0
DNA-DNA	0
RNA-RNA	0
DNA-RNA	0

Gene Ontology

Molecular Function

Accession	GO Term
GO:0003824	catalytic activity
GO:0008265	Mo-molybdopterin cofactor sulfurase activity
GO:0016740	transferase activity
GO:0016829	lyase activity
GO:0030151	molybdenum ion binding
GO:0030170	pyridoxal phosphate binding

Biological Process

GO:0006766	vitamin metabolic process
GO:0006767	water-soluble vitamin metabolic process
GO:0006777	Mo-molybdopterin cofactor biosynthetic process
GO:0008152	metabolic process
GO:0032324	molybdopterin cofactor biosynthetic process
GO:0043545	molybdopterin cofactor metabolic process
GO:0044281	small molecule metabolic process

Cellular Component

GO:0005575	cellular_component
GO:0005829	cytosol

Protein Structure and Domains

PDB ID

InterPro

IPR000192 Aminotransferase class V domain
IPR005302 Molybdenum cofactor sulfurase, C-terminal
IPR005303 MOSC, N-terminal beta barrel
IPR011037 Pyruvate kinase-like, insert domain
IPR015424 Pyridoxal phosphate-dependent transferase

PFAM

PF00266
PF03473
PF03476

PRINTS

PIRSF

SMART

TIGRFAMs

Post-translational Modifications

Modification

Cross-References

SwissProt

Q96EN8

PhosphoSite