MTARC2 (mitochondrial amidoxime reducing component 2)

Gene

• Entrez ID: 54996
• Gene name: Mitochondrial amidoxime reducing component 2
• Gene symbol: MTARC2
• Synonyms (NCBI Gene): MARC2, MOSC2
• Chromosome: 1
• Chromosome location: 1q41
• Summary: The protein encoded by this gene is an enzyme found in the outer mitochondrial membrane that reduces N-hydroxylated substrates. The encoded protein uses molybdenum as a cofactor and cytochrome b5 type B and NADH cytochrome b5 reductase as accessory protei

Gene ontology (GO)

GO ID	Ontology	Definition	Evidence	Reference
GO:0003824	Function	Catalytic activity	IEA
GO:0005739	Component	Mitochondrion	HTP	34800366
GO:0005739	Component	Mitochondrion	IDA
GO:0005739	Component	Mitochondrion	IEA
GO:0005739	Component	Mitochondrion	ISS
GO:0005741	Component	Mitochondrial outer membrane	IEA
GO:0005741	Component	Mitochondrial outer membrane	TAS
GO:0005777	Component	Peroxisome	IEA
GO:0006809	Process	Nitric oxide biosynthetic process	IDA	24500710
GO:0008940	Function	Nitrate reductase activity	IBA
GO:0008940	Function	Nitrate reductase activity	IDA	20861021
GO:0016020	Component	Membrane	IEA
GO:0016491	Function	Oxidoreductase activity	IEA
GO:0016661	Function	Oxidoreductase activity, acting on other nitrogenous compounds as donors	IMP	25713076
GO:0030151	Function	Molybdenum ion binding	IBA
GO:0030151	Function	Molybdenum ion binding	IDA	20861021
GO:0030151	Function	Molybdenum ion binding	IEA
GO:0030170	Function	Pyridoxal phosphate binding	IEA
GO:0042126	Process	Nitrate metabolic process	IBA
GO:0042126	Process	Nitrate metabolic process	IDA	20861021
GO:0043546	Function	Molybdopterin cofactor binding	IBA
GO:0043546	Function	Molybdopterin cofactor binding	IDA	20861021
GO:0051410	Process	Detoxification of nitrogen compound	NAS	20861021
GO:0070458	Process	Cellular detoxification of nitrogen compound	IMP	25713076
GO:0098809	Function	Nitrite reductase activity	IDA	24500710

Other IDs

• MIM: 614127
• HGNC: 26064
• e!Ensembl: ENSG00000117791

Protein

• UniProt ID: Q969Z3
• Protein name: Mitochondrial amidoxime reducing component 2 (mARC2) (EC 1.7.-.-) (Molybdenum cofactor sulfurase C-terminal domain-containing protein 2) (MOSC domain-containing protein 2) (Moco sulfurase C-terminal domain-containing protein 2)
• Protein function: Catalyzes the reduction of N-oxygenated molecules, acting as a counterpart of cytochrome P450 and flavin-containing monooxygenases in metabolic cycles (PubMed:21029045, PubMed:24423752). As a component of prodrug-converting system, reduces a mul
• Family and domains:

  Pfam

  Accession	ID	Position in sequence	Description	Type
  PF03476	MOSC_N	54 → 175	MOSC N-terminal beta barrel domain	Domain
  PF03473	MOSC	201 → 332	MOSC domain	Domain

• Sequence:

  MGASSSSALARLGLPARPWPRWLGVAALGLAAVALGTVAWRRAWPRRRRRLQQVGTVAKL
  WIYPVKSCKGVPVSEAECTAMGLRSGNLRDRFWLVIKEDGHMVTARQEPRLVLISIIYEN
  NCLIFRAPDMDQLVLPSKQPSSNKLHNCRIFGLDIKGRDCGNEAAKWFTNFLKTEAYRLV
  QFETNMKGRTSRKLLPTLDQNFQVAYPDYCPLLIMTDASLVDLNTRMEKKMKMENFRPNI
  VVTGCDAFEEDTWDELLIGSVEVKKVMACPRCILTTVDPDTGVIDRKQPLDTLKSYRLCD
  PSERELYKLSPLFGIYYSVEKIGSLRVGDPVYRMV

• Sequence length: 335

Pathways

Reactome:

Phase I - Functionalization of compounds

Associated diseases

Evidence Score:

• ★☆☆☆☆  Gene-disease association found in Text Mining only
• ★★☆☆☆  Found in Text Mining and Unknown/Other Associations
• ★★★☆☆  Reported in Unknown/Other Associations across ≥2 Sources
• ★★★★☆  ClinVar: Pathogenic/Likely Pathogenic (<5 Variants)
• ★★★★★  ClinVar: Pathogenic/Likely Pathogenic (≥5 Variants)

Unknown / Other Associations
Phenotype Name	Clinical Significance	Source	Evidence Score
AMPHETAMINE OR RELATED ACTING SYMPATHOMIMETIC ABUSE	—	Disgenet	★★☆☆☆ Found in Text Mining + Unknown/Other Associations
AMPHETAMINE-RELATED DISORDERS	—	CTD	★★☆☆☆ Found in Text Mining + Unknown/Other Associations
LEUKEMIA, PROMYELOCYTIC, ACUTE	—	CTD	★★☆☆☆ Found in Text Mining + Unknown/Other Associations