Entity Details

Primary name MOCS1_HUMAN
Entity type UniProt
Source Source Link

Details

AccessionQ9NZB8
EntryNameMOCS1_HUMAN
FullNameMolybdenum cofactor biosynthesis protein 1
TaxID9606
Evidenceevidence at protein level
Length636
SequenceStatuscomplete
DateCreated2003-05-09
DateModified2021-06-02

Ontological Relatives

GenesMOCS1

GO terms

Show/Hide Table
GOName
GO:0005525 GTP binding
GO:0005634 nucleus
GO:0005829 cytosol
GO:0006777 Mo-molybdopterin cofactor biosynthetic process
GO:0019008 molybdopterin synthase complex
GO:0032324 molybdopterin cofactor biosynthetic process
GO:0046872 metal ion binding
GO:0051539 4 iron, 4 sulfur cluster binding
GO:0061798 GTP 3',8'-cyclase activity
GO:0061799 cyclic pyranopterin monophosphate synthase activity

Subcellular Location

Show/Hide Table

Domains

Show/Hide Table
DomainNameCategoryType
IPR000385 MoaA/NifB/PqqE, iron-sulphur binding, conserved siteSiteConserved site
IPR002820 Molybdopterin cofactor biosynthesis C (MoaC) domainDomainDomain
IPR006638 Elp3/MiaB/NifBDomainDomain
IPR007197 Radical SAMDomainDomain
IPR010505 Molybdenum cofactor synthesis C-terminalDomainDomain
IPR013483 Molybdenum cofactor biosynthesis protein AFamilyFamily
IPR013785 Aldolase-type TIM barrelFamilyHomologous superfamily
IPR023045 Molybdenum cofactor biosynthesis CFamilyFamily
IPR036522 Molybdopterin cofactor biosynthesis C (MoaC) domain superfamilyFamilyHomologous superfamily

Diseases

Show/Hide Table
Disease IDSourceNameDescription
252150 OMIMMolybdenum cofactor deficiency, complementation group A (MOCODA)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. The disease is caused by variants affecting the gene represented in this entry.

Interactions

2 interactions

InteractorPartnerSourcesPublicationsLink
MOCS1_HUMANPIN1_HUMANBioGRID, IntAct21988832 details
MOCS1_HUMANPCNA_HUMANUniProt26030842 details