General Information of Protein (ID: PRT01609)
Name Cyclic pyranopterin monophosphate synthase (MOCS1)
Synonyms   Click to Show/Hide Synonyms of This Protein
Mocs1
Gene Name Mocs1 Gene ID
56738
UniProt ID
Q5RKZ7
Family Lyases (EC 4)
EC Number   EC: 4.1.99.22  (Click to Show/Hide the Complete EC Tree)
Lyases
Carbon-carbon lyase
Other carbon-carbon lyases
EC: 4.1.99.22
  Click to Show/Hide the Molecular/Functional Data (Sequence/Structure/Function) of This Protein
Sequence
MAARPAFGIVRQLLRSNARGCSSGAPVTQPRPGEPSRPTREGLSLRLQFLQEHAAPFSAF
LTDSFGRQHSYLRISLTEKCNLRCQYCMPEEGVPLTPKADLLTTEEILTLARLFVKEGVD
KIRLTGGEPLIRPDVVDIVARLHGLEGLRTIGLTTNGINLARLLPRLQQAGLNAVNISLD
TLVPAKFEFIVRRKGFHKVMEGIHKAIELGYKPVKVNCVVMRGLNEDELLDFVALTEGLP
LDVRFIEYMPFDGNKWNFKKMVSYKEMLDTIRQRWPGLEKLPEEDSSTAKAFKIPGFQGQ
ISFITSMSEHFCGTCNRLRITADGNLKVCLFGNSEVSLRDHLRAGASEEELLRIIGAAVG
RKKRQHAGMFNIAQMKNRPMILIGVLLMLQDSPPARWSNFSWDPLRVRNPSARQCLSDQM
ASLWKRHCIPKALPLSQQCLGSGSPQRHYSSYPDPDTHSKCLSTGSQAPDAPSGPGPTSN
QLTHVDSAGRASMVDVGGKPETERVAVASAMVLLGPVAFKLVQQNQLKKGDALVVAQLAG
VQAAKLTSQLIPLCHHVALSHVQVHLELDSTRHAVLIQASCRARGPTGVEMEALTSAAMA
ALTVYDMCKAVSRDIVVTEVKLISKTGGQRGDFHRA
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.
Regulatory Network
Full List of Metabolite(s) Regulated by This Protein
      Nucleosides, nucleotides, and analogues
            Molybdopterin Click to Show/Hide the Full List of Regulating Pair(s):   1 Pair(s)
               Detailed Information Metabo  Info click to show the details of this metabolite
               Regulating Pair Experim Info click to show the details of experiment for validating this pair [1]
                      Introduced Variation Knockout of Mocs1
                      Induced Change Molybdopterin concentration: decrease
                      Summary Introduced Variation         Induced Change 
                      Disease Status Organic acid disorderss [ICD-11: 5C50]
                      Details It is reported that knockout of Mocs1 leads to the decrease of molybdopterin levels compared with control group.
      Organic acids and derivatives
            Cysteine-S-sulfate Click to Show/Hide the Full List of Regulating Pair(s):   1 Pair(s)
               Detailed Information Metabo  Info click to show the details of this metabolite
               Regulating Pair Experim Info click to show the details of experiment for validating this pair [1]
                      Introduced Variation Knockout of Mocs1
                      Induced Change Cysteine-S-sulfate concentration: increase
                      Summary Introduced Variation         Induced Change 
                      Disease Status Organic acid disorderss [ICD-11: 5C50]
                      Details It is reported that knockout of Mocs1 leads to the increase of cysteine-S-sulfate levels compared with control group.
References
1 The pathogenesis of molybdenum cofactor deficiency, its delay by maternal clearance, and its expression pattern in microarray analysis. Mol Genet Metab. 2005 May;85(1):12-20.

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