Aliases for MTARC2 Gene
External Ids for MTARC2 Gene
Previous HGNC Symbols for MTARC2 Gene
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 proteins. One type of substrate used is N-hydroxylated nucleotide base analogues, which can be toxic to a cell. Other substrates include N(omega)-hydroxy-L-arginine (NOHA) and amidoxime prodrugs, which are activated by the encoded enzyme. Multiple transcript variants encoding the different isoforms have been found for this gene. [provided by RefSeq, Sep 2016]
GeneCards Summary for MTARC2 Gene
MTARC2 (Mitochondrial Amidoxime Reducing Component 2) is a Protein Coding gene. Diseases associated with MTARC2 include Epilepsy, Familial Adult Myoclonic, 3 and Epilepsy, Familial Adult Myoclonic, 2. Among its related pathways are Cytochrome P450 - arranged by substrate type and Metabolism. An important paralog of this gene is MTARC1.
UniProtKB/Swiss-Prot Summary for MTARC2 Gene
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 multitude of N-hydroxylated prodrugs particularly amidoximes, leading to increased drug bioavailability (PubMed:21029045, PubMed:24423752). May be involved in mitochondrial N(omega)-hydroxy-L-arginine (NOHA) reduction, regulating endogenous nitric oxide levels and biosynthesis (PubMed:21029045). Postulated to cleave the N-OH bond of N-hydroxylated substrates in concert with electron transfer from NADH to cytochrome b5 reductase then to cytochrome b5, the ultimate electron donor that primes the active site for substrate reduction (PubMed:21029045).