InnateDB: Systems Biology of the Innate Immune Response

Homo sapiens Protein: MOCS2

Summary

InnateDB Protein

IDBP-475125.4

Last Modified

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

Gene Symbol

MOCS2

Protein Name

molybdenum cofactor synthesis 2

Synonyms

MCBPE; MOCO1; MOCODB; MPTS;

Species

Homo sapiens

Ensembl Protein

ENSP00000424267

InnateDB Gene

IDBG-20420 (MOCS2)

Protein Structure

UniProt Annotation

Function

Acts as a sulfur carrier required for molybdopterin biosynthesis. Component of the molybdopterin synthase complex that catalyzes the conversion of precursor Z into molybdopterin by mediating the incorporation of 2 sulfur atoms into precursor Z to generate a dithiolene group. In the complex, serves as sulfur donor by being thiocarboxylated (-COSH) at its C-terminus by MOCS3. After interaction with MOCS2B, the sulfur is then transferred to precursor Z to form molybdopterin. {ECO:0000255HAMAP-Rule:MF_03051, ECO:0000269PubMed:12732628}.

Subcellular Localization

Cytoplasm, cytosol {ECO:0000255HAMAP- Rule:MF_03051, ECO:0000269PubMed:15073332}.

Disease Associations

Molybdenum cofactor deficiency, complementation group B (MOCODB) [MIM:252160]: An autosomal recessive metabolic disorder characterized by neonatal onset of intractable seizures, opisthotonus, and facial dysmorphism associated with hypouricemia and elevated urinary sulfite levels. Affected individuals show severe neurologic damage and often die in early childhood. {ECO:0000269PubMed:10053004, ECO:0000269PubMed:11746050, ECO:0000269PubMed:16021469, ECO:0000269PubMed:16737835}. Note=The disease is caused by mutations affecting the gene represented in this entry.

Tissue Specificity

Widely expressed. Highest levels are found in heart and skeletal muscle. Lower levels are present in brain, kidney and pancreas. Very low levels are found in lung and peripheral blood leukocytes. {ECO:0000269PubMed:10053003, ECO:0000269PubMed:9889283}.

Comments

Interactions

Number of Interactions

This gene and/or its encoded proteins are associated with 3 experimentally validated interaction(s) in this database.

Experimentally validated
Total	3 [view]
Protein-Protein	1 [view]
Protein-DNA	2 [view]
Protein-RNA	0
DNA-DNA	0
RNA-RNA	0
DNA-RNA	0

Gene Ontology

Molecular Function

Accession	GO Term
GO:0000166	nucleotide binding
GO:0030366	molybdopterin synthase activity

Biological Process

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

Cellular Component

GO:0005829	cytosol
GO:0019008	molybdopterin synthase complex
GO:0070062	extracellular vesicular exosome

Protein Structure and Domains

PDB ID

InterPro

IPR003749 ThiamineS/Molybdopterin converting factor subunit 1
IPR010034 Molybdopterin converting factor, subunit 1
IPR016155 Molybdopterin synthase/thiamin biosynthesis sulphur carrier, beta-grasp

PFAM

PF02597

PRINTS

PIRSF

SMART

TIGRFAMs

Post-translational Modifications

Modification

Cross-References

SwissProt

O96033

PhosphoSite