InnateDB: Systems Biology of the Innate Immune Response

Homo sapiens Protein: MOCS1

Summary

InnateDB Protein

IDBP-86283.6

Last Modified

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

Gene Symbol

MOCS1

Protein Name

molybdenum cofactor synthesis 1

Synonyms

MIG11; MOCOD; MOCODA;

Species

Homo sapiens

Ensembl Protein

ENSP00000344794

InnateDB Gene

IDBG-86275 (MOCS1)

Protein Structure

UniProt Annotation

Function

Isoform MOCS1A and isoform MOCS1B probably form a complex that catalyzes the conversion of 5'-GTP to cyclic pyranopterin monophosphate (cPMP or molybdopterin precursor Z). {ECO:0000269PubMed:11891227}.

Subcellular Localization

Disease Associations

Molybdenum cofactor deficiency, complementation group A (MOCODA) [MIM:252150]: An autosomal recessive metabolic disorder leading to the pleiotropic loss of molybdoenzyme activities. It is clinically characterized by onset in infancy of poor feeding, intractable seizures, severe psychomotor retardation, and death in early childhood in most patients. {ECO:0000269PubMed:12754701, ECO:0000269PubMed:16021469, ECO:0000269PubMed:9731530, ECO:0000269PubMed:9921896}. Note=The disease is caused by mutations affecting the gene represented in this entry.

Tissue Specificity

Isoform MOCS1A and isoform 2 are widely expressed. {ECO:0000269PubMed:12208140, ECO:0000269PubMed:9731530}.

Comments

Interactions

Number of Interactions

This gene and/or its encoded proteins are associated with 1 experimentally validated interaction(s) in this database.

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

Gene Ontology

Molecular Function

Accession	GO Term
GO:0003824	catalytic activity
GO:0005525	GTP binding
GO:0046872	metal ion binding
GO:0051536	iron-sulfur cluster binding
GO:0051539	4 iron, 4 sulfur cluster binding
GO:0061597	cyclic pyranopterin monophosphate 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:0005634	nucleus
GO:0005829	cytosol
GO:0019008	molybdopterin synthase complex

Protein Structure and Domains

PDB ID

InterPro

IPR002820 Molybdopterin cofactor biosynthesis C (MoaC) domain
IPR006638 Elongator protein 3/MiaB/NifB
IPR007197 Radical SAM
IPR010505 Molybdenum cofactor synthesis C-terminal
IPR013483 Molybdenum cofactor biosynthesis protein A
IPR023045 Molybdenum cofactor biosynthesis C

PFAM

PF01967
PF04055
PF06463

PRINTS

PIRSF

SMART

SM00729

TIGRFAMs

Post-translational Modifications

Modification

Cross-References

SwissProt

Q9NZB8