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Molybdenum cofactor (MoCo) is necessary for the function of all molybdoenzymes.
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This observation should encourage testing of additional intellectual disability patients with mild abnormalities of sulfite metabolism for MOCS3 mutations.
Among the associated variants were two in regions previously unreported for COPD; a low frequency non-synonymous SNP in MOCS3 (rs7269297, pdiscovery=3.08x10(-6), preplication=0.019) and a rare SNP in IFIT3, which emerged in the meta-analysis (rs140549288, pmeta=8.56x10(-6)).
ubiquitin-like Urm1.Uba4 systems are conserved and exchangeable between human and yeast cells
extension of the C terminus with an additional glycine of MOCS2A (show MOCS2 Proteins) and URM1 altered the localization of MOCS3 from the cytosol to the nucleus.
MOCS3 protein is believed to catalyze both the adenylation and the subsequent generation of a thiocarboxylate group at the C terminus of the smaller subunit of molybdopterin synthase
Electrospray ionization mass spectrometry performed on a rhodanese (show TST Proteins)-like carboxyl-terminal domain of human MOCS3 provides direct evidence for the formation of persulfide on cysteine residue 412.
in humans & most eukaryotes thiosulfate is not physiologic sulfur donor for MOCS3, whereas in bacterial homologs, which have arginine at last position of active site loop, thiosulfate can be used as a sulfur source for molybdenum cofactor biosynthesis.
The UBA4-URM1 system represents the evolutionary link between protein conjugation and protein modification by sulfur carrier proteins.
Nfs1 (show NFS1 Proteins) acts as a sulfur donor for MOCS3, a protein involved in molybdenum cofactor biosynthesis
Molybdenum cofactor (MoCo) is necessary for the function of all molybdoenzymes. The protein encoded by this gene adenylates and activates molybdopterin synthase, an enzyme required for biosynthesis of MoCo. This gene contains no introns. A pseudogene of this gene is present on chromosome 14.
molybdenum cofactor synthesis 3
, adenylyltransferase and sulfurtransferase MOCS3-like
, adenylyltransferase and sulfurtransferase MOCS3
, MPT synthase sulfurylase
, UBA4, ubiquitin-activating enzyme E1 homolog
, molybdenum cofactor synthesis protein 3
, molybdopterin synthase sulfurylase
, ubiquitin-like modifier activating enzyme 4
, Molybdenum cofactor synthesis protein 3