|Other Names||Molybdenum cofactor biosynthesis protein 1, Cell migration-inducing gene 11 protein, Molybdenum cofactor synthesis-step 1 protein A-B, Cyclic pyranopterin monophosphate synthase, Molybdenum cofactor biosynthesis protein A, Cyclic pyranopterin monophosphate synthase accessory protein, Molybdenum cofactor biosynthesis protein C, MOCS1|
|Format||Synthetic peptide was lyophilized with 100% acetonitrile and is supplied as a powder. Reconstitute with 0.1 ml DI water for a final concentration of 1 mg/ml.|
|Storage||Maintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C.|
|Precautions||This product is for research use only. Not for use in diagnostic or therapeutic procedures.|
|Function||Isoform MOCS1A and isoform MOCS1B probably form a complex that catalyzes the conversion of 5'-GTP to cyclic pyranopterin monophosphate (cPMP). MOCS1A catalyzes the cyclization of GTP to (8S)-3',8-cyclo-7,8-dihydroguanosine 5'- triphosphate and MOCS1B catalyzes the subsequent conversion of (8S)-3',8-cyclo-7,8-dihydroguanosine 5'-triphosphate to cPMP.|
|Tissue Location||Isoform MOCS1A and isoform 2 are widely expressed.|
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Molybdenum cofactor biosynthesis is a conserved pathwayleading to the biological activation of molybdenum. The proteinencoded by this gene is involved in this pathway. This gene wasoriginally thought to produce a bicistronic mRNA with the potentialto produce two proteins (MOCS1A and MOCS1B) from adjacent openreading frames. However, only the first open reading frame (MOCS1A)has been found to encode a protein from the putative bicistronicmRNA, whereas additional splice variants, whose full-length natureshave yet to be determined, are likely to produce a fusion betweenthe two open reading frames. This gene is defective in patientswith molybdenum cofactor deficiency, type A. A related pseudogenehas been identified on chromosome 16.
Sass, J.O., et al. Brain Dev. (2009) In press :Arenas, M., et al. J. Inherit. Metab. Dis. 32(4):560-569(2009)Ichida, K., et al. Nucleosides Nucleotides Nucleic Acids 25 (9-11), 1087-1091 (2006) :Macaya, A., et al. Neuropediatrics 36(6):389-394(2005)Leimkuhler, S., et al. Hum. Genet. 117(6):565-570(2005)
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