Showing Protein Molybdopterin synthase sulfur carrier subunit (HMDBP02015)

• 7471

1. MOCO1-A
2. MOCS2A
3. Molybdenum cofactor synthesis protein 2 small subunit
4. Molybdenum cofactor synthesis protein 2A
5. Molybdopterin-synthase small subunit
6. Sulfur carrier protein MOCS2A

• Folate biosynthesis
• molybdopterin biosynthesis
• Sulfur relay system

1. Cytoplasm
2. cytosol

>267 bp
ATGGTGCCGCTGTGCCAGGTTGAAGTATTGTATTTTGCAAAAAGTGCTGAAATAACAGGA
GTTCGTTCAGAGACCATTTCTGTGCCTCAAGAAATAAAAGCGTTGCAGCTGTGGAAGGAG
ATAGAAACTCGACATCCTGGATTGGCTGATGTTAGAAATCAGATAATATTTGCTGTTCGT
CAAGAATATGTCGAGCTTGGAGATCAGCTCCTCGTGCTTCAGCCTGGAGACGAAATTGCC
GTTATCCCCCCCATTAGTGGAGGATAG

• ThiS (PF02597 )

>Molybdopterin synthase sulfur carrier subunit
MVPLCQVEVLYFAKSAEITGVRSETISVPQEIKALQLWKEIETRHPGLADVRNQIIFAVR
QEYVELGDQLLVLQPGDEIAVIPPISGG

1. Stallmeyer B, Drugeon G, Reiss J, Haenni AL, Mendel RR: Human molybdopterin synthase gene: identification of a bicistronic transcript with overlapping reading frames. Am J Hum Genet. 1999 Mar;64(3):698-705. [PubMed:10053003 ]
2. Sloan J, Kinghorn JR, Unkles SE: The two subunits of human molybdopterin synthase: evidence for a bicistronic messenger RNA with overlapping reading frames. Nucleic Acids Res. 1999 Feb 1;27(3):854-8. [PubMed:9889283 ]
3. Leimkuhler S, Freuer A, Araujo JA, Rajagopalan KV, Mendel RR: Mechanistic studies of human molybdopterin synthase reaction and characterization of mutants identified in group B patients of molybdenum cofactor deficiency. J Biol Chem. 2003 Jul 11;278(28):26127-34. Epub 2003 May 5. [PubMed:12732628 ]
4. Matthies A, Rajagopalan KV, Mendel RR, Leimkuhler S: Evidence for the physiological role of a rhodanese-like protein for the biosynthesis of the molybdenum cofactor in humans. Proc Natl Acad Sci U S A. 2004 Apr 20;101(16):5946-51. Epub 2004 Apr 8. [PubMed:15073332 ]
5. Matthies A, Nimtz M, Leimkuhler S: Molybdenum cofactor biosynthesis in humans: identification of a persulfide group in the rhodanese-like domain of MOCS3 by mass spectrometry. Biochemistry. 2005 May 31;44(21):7912-20. [PubMed:15910006 ]
6. Krepinsky K, Leimkuhler S: Site-directed mutagenesis of the active site loop of the rhodanese-like domain of the human molybdopterin synthase sulfurase MOCS3. Major differences in substrate specificity between eukaryotic and bacterial homologs. FEBS J. 2007 Jun;274(11):2778-87. Epub 2007 Apr 25. [PubMed:17459099 ]
7. Schmitz J, Chowdhury MM, Hanzelmann P, Nimtz M, Lee EY, Schindelin H, Leimkuhler S: The sulfurtransferase activity of Uba4 presents a link between ubiquitin-like protein conjugation and activation of sulfur carrier proteins. Biochemistry. 2008 Jun 17;47(24):6479-89. doi: 10.1021/bi800477u. Epub 2008 May 21. [PubMed:18491921 ]
8. Reiss J, Dorche C, Stallmeyer B, Mendel RR, Cohen N, Zabot MT: Human molybdopterin synthase gene: genomic structure and mutations in molybdenum cofactor deficiency type B. Am J Hum Genet. 1999 Mar;64(3):706-11. [PubMed:10053004 ]
9. Johnson JL, Coyne KE, Rajagopalan KV, Van Hove JL, Mackay M, Pitt J, Boneh A: Molybdopterin synthase mutations in a mild case of molybdenum cofactor deficiency. Am J Med Genet. 2001 Nov 22;104(2):169-73. [PubMed:11746050 ]
10. Leimkuhler S, Charcosset M, Latour P, Dorche C, Kleppe S, Scaglia F, Szymczak I, Schupp P, Hahnewald R, Reiss J: Ten novel mutations in the molybdenum cofactor genes MOCS1 and MOCS2 and in vitro characterization of a MOCS2 mutation that abolishes the binding ability of molybdopterin synthase. Hum Genet. 2005 Oct;117(6):565-70. Epub 2005 Jul 14. [PubMed:16021469 ]
11. Hahnewald R, Leimkuhler S, Vilaseca A, Acquaviva-Bourdain C, Lenz U, Reiss J: A novel MOCS2 mutation reveals coordinated expression of the small and large subunit of molybdopterin synthase. Mol Genet Metab. 2006 Nov;89(3):210-3. Epub 2006 Jun 5. [PubMed:16737835 ]