Entity Details

Primary name MOC2A_HUMAN
Entity type UniProt
Source Source Link

Details

AccessionO96033
EntryNameMOC2A_HUMAN
FullNameMolybdopterin synthase sulfur carrier subunit
TaxID9606
Evidenceevidence at protein level
Length88
SequenceStatuscomplete
DateCreated2002-02-11
DateModified2021-06-02

Ontological Relatives

GenesMOCS2

GO terms

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GOName
GO:0000166 nucleotide binding
GO:0005829 cytosol
GO:0006777 Mo-molybdopterin cofactor biosynthetic process
GO:0019008 molybdopterin synthase complex
GO:0030366 molybdopterin synthase activity
GO:0032324 molybdopterin cofactor biosynthetic process

Subcellular Location

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Subcellular Location
Cytoplasm

Domains

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DomainNameCategoryType
IPR003749 Sulfur carrier ThiS/MoaD-likeFamilyFamily
IPR012675 Beta-grasp domain superfamilyFamilyHomologous superfamily
IPR016155 Molybdopterin synthase/thiamin biosynthesis sulphur carrier, beta-graspFamilyHomologous superfamily
IPR028887 Molybdopterin synthase sulfur carrier subunit, eukaryoteFamilyFamily
IPR044672 Molybdopterin synthase sulfur carrier subunitFamilyFamily

Diseases

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Disease IDSourceNameDescription
252160 OMIMMolybdenum cofactor deficiency, complementation group B (MOCODB)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. The disease is caused by variants affecting the gene represented in this entry.

Interactions

4 interactions