Biology:Mitochondrial amidoxime reducing component 1

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Short description: Mammalian protein found in Homo sapiens


A representation of the 3D structure of the protein myoglobin showing turquoise α-helices.
Generic protein structure example

Mitochondrial amidoxime-reducing component 1 (also known as MOCO sulphurase C-terminal domain containing 1, MOSC1 or MARC1) is a mammalian molybdenum-containing enzyme. It is located in the outer mitochondrial membrane and consists of a N-terminal mitochondrial signal domain facing the inter-membrane space, a transmembrane domain, and a C-terminal catalytic domain facing the cytosol.[1] In humans it is encoded by the MOSC1 gene.[2][3]

MOCO stands for molybdenum cofactor.

MOSC1 has been reported to reduce amidoximes to amidines.[4][5]

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.[6] A genome-wide association study involving subjects from the UK Biobank further established as association of alcoholic-related liver disease.[7]

See also

References

  1. "The mitochondrial amidoxime-reducing component (mARC1) is a novel signal-anchored protein of the outer mitochondrial membrane". The Journal of Biological Chemistry 287 (51): 42795–42803. December 2012. doi:10.1074/jbc.M112.419424. PMID 23086957. 
  2. "MOSC domains: ancient, predicted sulfur-carrier domains, present in diverse metal-sulfur cluster biosynthesis proteins including Molybdenum cofactor sulfurases". FEMS Microbiology Letters 207 (1): 55–61. January 2002. doi:10.1016/S0378-1097(01)00515-8. PMID 11886751. 
  3. "Entrez Gene: Mitochondrial amidoxime reducing component 1". https://www.ncbi.nlm.nih.gov/gene/64757. 
  4. "Identification of the missing component in the mitochondrial benzamidoxime prodrug-converting system as a novel molybdenum enzyme". The Journal of Biological Chemistry 281 (46): 34796–34802. November 2006. doi:10.1074/jbc.M607697200. PMID 16973608. 
  5. "The fourth molybdenum containing enzyme mARC: cloning and involvement in the activation of N-hydroxylated prodrugs". Journal of Medicinal Chemistry 51 (24): 8173–8177. December 2008. doi:10.1021/jm8010417. PMID 19053771. 
  6. "A missense variant in Mitochondrial Amidoxime Reducing Component 1 gene and protection against liver disease". PLoS Genetics 16 (4): e1008629. April 2020. doi:10.1371/journal.pgen.1008629. PMID 32282858. 
  7. "MARC1 and HNRNPUL1: Two Novel Players in Alcohol-related Liver Disease" (in English). Gastroenterology 159 (4): 1231–1232. October 2020. doi:10.1053/j.gastro.2020.08.009. PMID 32800779.