Mitochondrial amidoxime reducing component 1

Mitochondrial amidoxime-reducing component 1 (also known as MOCO sulphurase C-terminal domain containing 1, MOSC1 or MARC1) is an enzyme which in humans is encoded by the MOSC1 gene.[5][6]

MTARC1
Identifiers
Aliases	MTARC1, MOSC1, mitochondrial amidoxime reducing component 1, MARC1
External IDs	OMIM: 614126 MGI: 1913362 HomoloGene: 129604 GeneCards: MTARC1
Gene location (Human) Chr. Chromosome 1 (human)[1] Band 1q41 Start 220,786,913 bp[1] End 220,819,659 bp[1]
Gene location (Mouse) Chr. Chromosome 1 (mouse)[2] Band 1|1 H5 Start 184,786,767 bp[2] End 184,811,313 bp[2]
Gene ontology Molecular function • molybdopterin cofactor binding • molybdenum ion binding • oxidoreductase activity • pyridoxal phosphate binding • nitrate reductase activity • catalytic activity Cellular component • integral component of membrane • membrane • mitochondrion • mitochondrial outer membrane Biological process • detoxification of nitrogen compound • oxidation-reduction process • nitrate metabolic process • xenobiotic metabolic process • metabolism Sources:Amigo / QuickGO
Orthologs
Species	Human	Mouse
Entrez	64757	66112
Ensembl	ENSG00000186205	ENSMUSG00000026621
UniProt	Q5VT66	Q9CW42
RefSeq (mRNA)	NM_022746	NM_001081361 NM_001290273
RefSeq (protein)	NP_073583	NP_001277202
Location (UCSC)	Chr 1: 220.79 – 220.82 Mb	Chr 1: 184.79 – 184.81 Mb
PubMed search	[3]	[4]
Wikidata
View/Edit Human View/Edit Mouse

MOCO stands for molybdenum cofactor.

MOSC1 has been reported to reduce amidoximes to amidines.[7][8]

Genetic variation in MARC1 has been reported to be associated with lower blood cholesterol levels, blood liver enzyme levels, reduced liver fat and protection from cirrhosis suggesting that MARC1 deficiency may protect against liver disease.[9]

See also

• MOCOS

References

1. GRCh38: Ensembl release 89: ENSG00000186205 - Ensembl, May 2017
2. GRCm38: Ensembl release 89: ENSMUSG00000026621 - Ensembl, May 2017
3. "Human PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
4. "Mouse PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
5. Anantharaman V, Aravind L (January 2002). "MOSC domains: ancient, predicted sulfur-carrier domains, present in diverse metal-sulfur cluster biosynthesis proteins including Molybdenum cofactor sulfurases". FEMS Microbiol. Lett. 207 (1): 55–61. doi:10.1016/S0378-1097(01)00515-8. PMID 11886751.
6. "Entrez Gene: Mitochondrial amidoxime reducing component 1". Retrieved 2016-03-03.
7. Havemeyer A, Bittner F, Wollers S, Mendel R, Kunze T, Clement B (November 2006). "Identification of the missing component in the mitochondrial benzamidoxime prodrug-converting system as a novel molybdenum enzyme". J. Biol. Chem. 281 (46): 34796–802. doi:10.1074/jbc.M607697200. PMID 16973608.
8. Gruenewald S, Wahl B, Bittner F, Hungeling H, Kanzow S, Kotthaus J, Schwering U, Mendel RR, Clement B (December 2008). "The fourth molybdenum containing enzyme mARC: cloning and involvement in the activation of N-hydroxylated prodrugs". J. Med. Chem. 51 (24): 8173–7. doi:10.1021/jm8010417. PMID 19053771.
9. Kathiresan, Sekar; Gabriel, Stacey; Denny, Joshua; Daly, Mark; Saleheen, Danish; Danesh, John; Gupta, Namrata; Erdmann, Jeanette; Baber, Usman (2019-03-31). "A missense variant in Mitochondrial Amidoxime Reducing Component 1 gene and protection against liver disease". bioRxiv: 594523. doi:10.1101/594523.