| Identification |
|---|
| HMDB Protein ID
| HMDBP02015 |
| Secondary Accession Numbers
| |
| Name
| Molybdopterin synthase sulfur carrier subunit |
| Synonyms
|
- MOCO1-A
- MOCS2A
- Molybdenum cofactor synthesis protein 2 small subunit
- Molybdenum cofactor synthesis protein 2A
- Molybdopterin-synthase small subunit
- Sulfur carrier protein MOCS2A
|
| Gene Name
| MOCS2 |
| Protein Type
| Enzyme |
| Biological Properties |
|---|
| General Function
| Involved in Mo-molybdopterin cofactor biosynthetic process |
| Specific Function
| Acts as a sulfur carrier required for molybdopterin biosynthesis. Component of the molybdopterin synthase complex that catalyzes the conversion of precursor Z into molybdopterin by mediating the incorporation of 2 sulfur atoms into precursor Z to generate a dithiolene group. In the complex, serves as sulfur donor by being thiocarboxylated (-COSH) at its C-terminus by MOCS3. After interaction with MOCS2B, the sulfur is then transferred to precursor Z to form molybdopterin.
|
| Pathways
|
- Folate biosynthesis
- molybdopterin biosynthesis
- Sulfur relay system
|
| Reactions
|
| Cyclic pyranopterin monophosphate + Sulfur donor → Molybdopterin |
details
|
|
| GO Classification
|
| Biological Process |
| water-soluble vitamin metabolic process |
| Mo-molybdopterin cofactor biosynthetic process |
| Cellular Component |
| cytosol |
| molybdopterin synthase complex |
| Molecular Function |
| nucleotide binding |
| Process |
| metabolic process |
| coenzyme biosynthetic process |
| sulfur metabolic process |
| cellular metabolic process |
| cofactor metabolic process |
| coenzyme metabolic process |
| mo-molybdopterin cofactor biosynthetic process |
|
| Cellular Location
|
- Cytoplasm
- cytosol
|
| Gene Properties |
|---|
| Chromosome Location
| 5 |
| Locus
| 5q11 |
| SNPs
| MOCS2 |
| Gene Sequence
|
>267 bp
ATGGTGCCGCTGTGCCAGGTTGAAGTATTGTATTTTGCAAAAAGTGCTGAAATAACAGGA
GTTCGTTCAGAGACCATTTCTGTGCCTCAAGAAATAAAAGCGTTGCAGCTGTGGAAGGAG
ATAGAAACTCGACATCCTGGATTGGCTGATGTTAGAAATCAGATAATATTTGCTGTTCGT
CAAGAATATGTCGAGCTTGGAGATCAGCTCCTCGTGCTTCAGCCTGGAGACGAAATTGCC
GTTATCCCCCCCATTAGTGGAGGATAG
|
| Protein Properties |
|---|
| Number of Residues
| 88 |
| Molecular Weight
| 9755.235 |
| Theoretical pI
| 4.702 |
| Pfam Domain Function
|
|
| Signals
|
Not Available
|
|
Transmembrane Regions
|
Not Available
|
| Protein Sequence
|
>Molybdopterin synthase sulfur carrier subunit
MVPLCQVEVLYFAKSAEITGVRSETISVPQEIKALQLWKEIETRHPGLADVRNQIIFAVR
QEYVELGDQLLVLQPGDEIAVIPPISGG
|
| External Links |
|---|
| GenBank ID Protein
| 4262372 |
| UniProtKB/Swiss-Prot ID
| O96033 |
| UniProtKB/Swiss-Prot Entry Name
| MOC2A_HUMAN |
| PDB IDs
|
Not Available |
| GenBank Gene ID
| AF091871 |
| GeneCard ID
| MOCS2 |
| GenAtlas ID
| MOCS2 |
| HGNC ID
| HGNC:7193 |
| References |
|---|
| General References
| - 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 ]
- 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 ]
- 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 ]
- 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 ]
- 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 ]
- 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 ]
- 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 ]
- 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 ]
- 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 ]
- 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 ]
- 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 ]
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