MOCS1

MOCS1
Identifiers
Aliases	MOCS1, MIG11, MOCOD, MOCODA, molybdenum cofactor synthesis 1, MOCS1A, MOCS1B
External IDs	OMIM: 603707; MGI: 1928904; HomoloGene: 129502; GeneCards: MOCS1; OMA:MOCS1 - orthologs
Gene location (Human)
Chr.	Chromosome 6 (human)
End	39,934,551 bp
Gene location (Mouse)
Chr.	Chromosome 17 (mouse)
End	49,762,463 bp
RNA expression pattern
	Top expressed in
	apex of heart; ; popliteal artery; ; tibial arteries; ; right lung; ; pancreatic ductal cell; ; right lobe of liver; ; subcutaneous adipose tissue; ; mucosa of transverse colon; ; gastrocnemius muscle; ; tibial nerve;
	Top expressed in
	granulocyte; ; muscle of thigh; ; tail of embryo; ; lip; ; genital tubercle; ; right kidney; ; esophagus; ; ascending aorta; ; left lobe of liver; ; duodenum;
	More reference expression data
	; ;
	More reference expression data
Gene ontology
Molecular function	metal ion binding; nucleotide binding; 4 iron, 4 sulfur cluster binding; catalytic activity; lyase activity; GTP binding; iron-sulfur cluster binding; GTP 3',8'-cyclase activity; cyclic pyranopterin monophosphate synthase activity;
Cellular component	molybdopterin synthase complex; cytosol; nucleus;
Biological process	Mo-molybdopterin cofactor biosynthetic process; molybdopterin cofactor biosynthetic process;
	Sources:Amigo / QuickGO
Orthologs
	4337
	56738
	ENSG00000124615
	ENSMUSG00000064120
	Q9NZB8
	Q5RKZ7
NM_001075098; NM_005942; NM_005943; NM_138928; NM_001358529;
	NM_001358530; NM_001358531; NM_001358533; NM_001358534
	NM_020042; NM_028464
NP_001068566; NP_005934; NP_001345458; NP_001345459; NP_001345460;
	NP_001345462; NP_001345463
	NP_064426; NP_082740
	Wikidata
View/Edit Human	View/Edit Mouse

Molybdenum cofactor biosynthesis protein 1 is a protein that in humans and other animals, fungi, and cellular slime molds, is encoded by the MOCS1 gene.

Both copies of this gene are defective in patients with molybdenum cofactor deficiency, type A.

Molybdenum cofactor biosynthesis is a conserved pathway leading to the biological activation of molybdenum. The gene contains two open reading frames, termed MOCS1A and MOCS1B. The former is a GTP 3',8-cyclase homologous to MoaA, the latter a cyclic pyranopterin monophosphate synthase homologous to MoaC. The two work in series in the biosynthetic pathway.

The gene has a complex pattern of alternative splicing. MOCS1A is produced by a splice variant that appears to be a bicistronic mRNA containing both ORFs, but this mRNA actually only translates to MOCS1A. Two of the splice variants found for this gene express proteins (MOCS1A-MOCS1B) that result from a fusion between the two open reading frames; active MOCS1B is only produced in a form preceded by an inactive version of MOCS1A.