Mitochondrial amidoxime reducing component 1 explained

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

Notes and References

  1. Klein JM, Busch JD, Potting C, Baker MJ, Langer T, Schwarz G . 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 . 23086957 . 3525010 . 10.1074/jbc.M112.419424 . free .
  2. Anantharaman V, Aravind L . 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 . 11886751 . 10.1016/S0378-1097(01)00515-8 .
  3. Web site: Entrez Gene: Mitochondrial amidoxime reducing component 1 . 2016-03-03 .
  4. Havemeyer A, Bittner F, Wollers S, Mendel R, Kunze T, Clement B . 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 . 16973608 . 10.1074/jbc.M607697200 . free .
  5. Gruenewald S, Wahl B, Bittner F, Hungeling H, Kanzow S, Kotthaus J, Schwering U, Mendel RR, Clement B . 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 . 19053771 . 10.1021/jm8010417 .
  6. Emdin CA, Haas ME, Khera AV, Aragam K, Chaffin M, Klarin D, Hindy G, Jiang L, Wei WQ, Feng Q, Karjalainen J, Havulinna A, Kiiskinen T, Bick A, Ardissino D, Wilson JG, Schunkert H, McPherson R, Watkins H, Elosua R, Bown MJ, Samani NJ, Baber U, Erdmann J, Gupta N, Danesh J, Saleheen D, Chang KM, Vujkovic M, Voight B, Damrauer S, Lynch J, Kaplan D, Serper M, Tsao P, Mercader J, Hanis C, Daly M, Denny J, Gabriel S, Kathiresan S . A missense variant in Mitochondrial Amidoxime Reducing Component 1 gene and protection against liver disease . PLOS Genetics . 16 . 4 . e1008629 . April 2020 . 32282858 . 7200007 . 10.1371/journal.pgen.1008629 . free . 10.1101/594523 .
  7. Romeo S . MARC1 and HNRNPUL1: Two Novel Players in Alcohol-related Liver Disease . English . Gastroenterology . 159 . 4 . 1231–1232 . October 2020 . 32800779 . 10.1053/j.gastro.2020.08.009 . free .

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