UniProtKB - Q969Z3 (MARC2_HUMAN)
Mitochondrial amidoxime reducing component 2
MTARC2
Functioni
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).
2 PublicationsCatalytic activityi
- 2 Fe(II)-[cytochrome b5] + 2 H+ + Nω-hydroxy-L-arginine = 2 Fe(III)-[cytochrome b5] + H2O + L-arginine1 PublicationThis reaction proceeds in the forward1 Publication direction.
Cofactori
Kineticsi
- KM=830 µM for benzamidoxime2 Publications
- KM=400 µM for benzamidoxime (at pH 6.0 and 37 degrees Celsius)1 Publication
- KM=3 mM for NOHA2 Publications
- KM=3.7 mM for NHAM2 Publications
- Vmax=307 nmol/min/mg enzyme toward benzamidoxime2 Publications
- Vmax=119 nmol/min/mg enzyme toward benzamidoxime (at pH 6.0 and 37 degrees Celsius)1 Publication
- Vmax=373 nmol/min/mg enzyme toward NOHA2 Publications
- Vmax=36.5 nmol/min/mg enzyme toward NHAM2 Publications
GO - Molecular functioni
- molybdenum ion binding Source: UniProtKB
- molybdopterin cofactor binding Source: UniProtKB
- nitrate reductase activity Source: UniProtKB
- nitrite reductase activity Source: FlyBase
- oxidoreductase activity, acting on other nitrogenous compounds as donors Source: FlyBase
- pyridoxal phosphate binding Source: InterPro
GO - Biological processi
- cellular detoxification of nitrogen compound Source: FlyBase
- detoxification of nitrogen compound Source: UniProtKB
- nitrate metabolic process Source: UniProtKB
- nitric oxide biosynthetic process Source: FlyBase
Keywordsi
Molecular function | Oxidoreductase |
Ligand | Molybdenum |
Enzyme and pathway databases
BRENDAi | 1.7.2.1, 2681 |
PathwayCommonsi | Q969Z3 |
Reactomei | R-HSA-211945, Phase I - Functionalization of compounds |
SABIO-RKi | Q969Z3 |
SignaLinki | Q969Z3 |
Names & Taxonomyi
Protein namesi | Recommended name: Mitochondrial amidoxime reducing component 21 Publication (EC:1.7.-.-)Short name: mARC21 Publication Alternative name(s): Molybdenum cofactor sulfurase C-terminal domain-containing protein 2 Short name: MOSC domain-containing protein 2 Short name: Moco sulfurase C-terminal domain-containing protein 2 |
Gene namesi | |
Organismi | Homo sapiens (Human) |
Taxonomic identifieri | 9606 [NCBI] |
Taxonomic lineagei | Eukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Mammalia › Eutheria › Euarchontoglires › Primates › Haplorrhini › Catarrhini › Hominidae › Homo |
Proteomesi |
|
Organism-specific databases
HGNCi | HGNC:26064, MTARC2 |
MIMi | 614127, gene |
neXtProti | NX_Q969Z3 |
VEuPathDBi | HostDB:ENSG00000117791 |
Subcellular locationi
Mitochondrion
Peroxisome
- Peroxisome By similarity
Mitochondrion
- mitochondrial outer membrane Source: Reactome
- mitochondrion Source: LIFEdb
Peroxisome
- peroxisome Source: UniProtKB-SubCell
Keywords - Cellular componenti
Membrane, Mitochondrion, Mitochondrion outer membrane, PeroxisomePathology & Biotechi
Organism-specific databases
DisGeNETi | 54996 |
OpenTargetsi | ENSG00000117791 |
PharmGKBi | PA142671345 |
Miscellaneous databases
Pharosi | Q969Z3, Tbio |
Chemistry databases
ChEMBLi | CHEMBL4523423 |
Genetic variation databases
BioMutai | MARC2 |
DMDMi | 74760692 |
PTM / Processingi
Molecule processing
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Transit peptidei | 1 – 35 | MitochondrionSequence analysisAdd BLAST | 35 | |
ChainiPRO_0000273340 | 36 – 335 | Mitochondrial amidoxime reducing component 2Add BLAST | 300 |
Amino acid modifications
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Cross-linki | 59 | Glycyl lysine isopeptide (Lys-Gly) (interchain with G-Cter in ubiquitin)1 Publication | ||
Cross-linki | 138 | Glycyl lysine isopeptide (Lys-Gly) (interchain with G-Cter in ubiquitin)1 Publication | ||
Cross-linki | 144 | Glycyl lysine isopeptide (Lys-Gly) (interchain with G-Cter in ubiquitin)1 Publication | ||
Modified residuei | 156 | N6-acetyllysine; alternateBy similarity | 1 | |
Cross-linki | 156 | Glycyl lysine isopeptide (Lys-Gly) (interchain with G-Cter in ubiquitin); alternate1 Publication | ||
Cross-linki | 166 | Glycyl lysine isopeptide (Lys-Gly) (interchain with G-Cter in ubiquitin)1 Publication | ||
Cross-linki | 173 | Glycyl lysine isopeptide (Lys-Gly) (interchain with G-Cter in ubiquitin)1 Publication | ||
Cross-linki | 187 | Glycyl lysine isopeptide (Lys-Gly) (interchain with G-Cter in ubiquitin)1 Publication | ||
Cross-linki | 287 | Glycyl lysine isopeptide (Lys-Gly) (interchain with G-Cter in ubiquitin)1 Publication | ||
Cross-linki | 294 | Glycyl lysine isopeptide (Lys-Gly) (interchain with G-Cter in ubiquitin)1 Publication |
Post-translational modificationi
Keywords - PTMi
Acetylation, Isopeptide bond, Ubl conjugationProteomic databases
EPDi | Q969Z3 |
jPOSTi | Q969Z3 |
MassIVEi | Q969Z3 |
MaxQBi | Q969Z3 |
PaxDbi | Q969Z3 |
PeptideAtlasi | Q969Z3 |
PRIDEi | Q969Z3 |
ProteomicsDBi | 75882 [Q969Z3-1] 75883 [Q969Z3-2] |
PTM databases
iPTMneti | Q969Z3 |
PhosphoSitePlusi | Q969Z3 |
Expressioni
Gene expression databases
Bgeei | ENSG00000117791, Expressed in left lobe of thyroid gland and 224 other tissues |
ExpressionAtlasi | Q969Z3, baseline and differential |
Genevisiblei | Q969Z3, HS |
Organism-specific databases
HPAi | ENSG00000117791, Tissue enhanced (kidney, liver) |
Interactioni
Subunit structurei
Component of a complex composed of cytochrome b5, NADH-cytochrome b5 reductase (CYB5R3) and MTARC2.
By similarityProtein-protein interaction databases
BioGRIDi | 120329, 36 interactors |
IntActi | Q969Z3, 10 interactors |
MINTi | Q969Z3 |
STRINGi | 9606.ENSP00000355880 |
Miscellaneous databases
RNActi | Q969Z3, protein |
Family & Domainsi
Domains and Repeats
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Domaini | 188 – 334 | MOSCPROSITE-ProRule annotationAdd BLAST | 147 |
Keywords - Domaini
Transit peptidePhylogenomic databases
eggNOGi | KOG2362, Eukaryota |
GeneTreei | ENSGT00940000159665 |
HOGENOMi | CLU_028286_6_2_1 |
InParanoidi | Q969Z3 |
OMAi | GYPFLLI |
OrthoDBi | 919343at2759 |
PhylomeDBi | Q969Z3 |
TreeFami | TF316807 |
Family and domain databases
InterProi | View protein in InterPro IPR005302, MoCF_Sase_C IPR005303, MOSC_N IPR011037, Pyrv_Knase-like_insert_dom_sf |
Pfami | View protein in Pfam PF03473, MOSC, 1 hit PF03476, MOSC_N, 1 hit |
SUPFAMi | SSF50800, SSF50800, 1 hit |
PROSITEi | View protein in PROSITE PS51340, MOSC, 1 hit |
s (2+)i Sequence
Sequence statusi: Complete.
: The displayed sequence is further processed into a mature form. Sequence processingi
This entry describes 2 produced by isoformsialternative splicing. AlignAdd to basketThis entry has 2 described isoforms and 2 potential isoforms that are computationally mapped.Show allAlign All
This isoform has been chosen as the sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry. canonicali
10 20 30 40 50
MGASSSSALA RLGLPARPWP RWLGVAALGL AAVALGTVAW RRAWPRRRRR
60 70 80 90 100
LQQVGTVAKL WIYPVKSCKG VPVSEAECTA MGLRSGNLRD RFWLVIKEDG
110 120 130 140 150
HMVTARQEPR LVLISIIYEN NCLIFRAPDM DQLVLPSKQP SSNKLHNCRI
160 170 180 190 200
FGLDIKGRDC GNEAAKWFTN FLKTEAYRLV QFETNMKGRT SRKLLPTLDQ
210 220 230 240 250
NFQVAYPDYC PLLIMTDASL VDLNTRMEKK MKMENFRPNI VVTGCDAFEE
260 270 280 290 300
DTWDELLIGS VEVKKVMACP RCILTTVDPD TGVIDRKQPL DTLKSYRLCD
310 320 330
PSERELYKLS PLFGIYYSVE KIGSLRVGDP VYRMV
Computationally mapped potential isoform sequencesi
There are 2 potential isoforms mapped to this entry.BLASTAlignShow allAdd to basketX1WI34 | X1WI34_HUMAN | Mitochondrial amidoxime reducing co... | MTARC2 | 172 | Annotation score: | ||
F6V6Z1 | F6V6Z1_HUMAN | Mitochondrial amidoxime reducing co... | MTARC2 | 94 | Annotation score: |
Sequence cautioni
Experimental Info
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Sequence conflicti | 169 | T → A in CAL38507 (PubMed:17974005).Curated | 1 |
Natural variant
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Natural variantiVAR_062275 | 3 | A → S1 PublicationCorresponds to variant dbSNP:rs72472370Ensembl. | 1 | |
Natural variantiVAR_030133 | 244 | G → S Decreased catalytic efficiency toward benzamidoxime; no effect on affinity for benzamidoxime; no effect on binding of the molybdenum cofactor. 2 PublicationsCorresponds to variant dbSNP:rs3795535Ensembl. | 1 | |
Natural variantiVAR_070777 | 245 | C → W Decreased catalytic activity toward benzamidoxime; no effect on affinity for benzamidoxime; no effect on binding of the molybdenum cofactor. 1 PublicationCorresponds to variant dbSNP:rs76664695Ensembl. | 1 |
Alternative sequence
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Alternative sequenceiVSP_022513 | 251 – 257 | DTWDELL → ASATRRD in isoform 2. 1 Publication | 7 | |
Alternative sequenceiVSP_022514 | 258 – 335 | Missing in isoform 2. 1 PublicationAdd BLAST | 78 |
Sequence databases
Select the link destinations: EMBLi GenBanki DDBJi Links Updated | AL136931 mRNA Translation: CAB66865.1 AM393631 mRNA Translation: CAL38507.1 EU567145 Genomic DNA Translation: ACB21048.1 AL359353 Genomic DNA No translation available. AL606726 Genomic DNA No translation available. CH471100 Genomic DNA Translation: EAW93293.1 CH471100 Genomic DNA Translation: EAW93294.1 BC011973 mRNA Translation: AAH11973.1 BC015829 mRNA Translation: AAH15829.2 BC016859 mRNA Translation: AAH16859.1 AK000612 mRNA Translation: BAA91287.1 Different initiation. |
CCDSi | CCDS1525.1 [Q969Z3-1] CCDS81425.1 [Q969Z3-2] |
RefSeqi | NP_001304267.1, NM_001317338.1 [Q969Z3-1] NP_001317971.1, NM_001331042.1 [Q969Z3-2] NP_060368.2, NM_017898.4 [Q969Z3-1] |
Genome annotation databases
Ensembli | ENST00000359316.6; ENSP00000352266.2; ENSG00000117791.16 [Q969Z3-2] ENST00000366913.8; ENSP00000355880.3; ENSG00000117791.16 |
GeneIDi | 54996 |
KEGGi | hsa:54996 |
MANE-Selecti | ENST00000366913.8; ENSP00000355880.3; NM_017898.5; NP_060368.2 |
UCSCi | uc001hmq.4, human [Q969Z3-1] |
Keywords - Coding sequence diversityi
Alternative splicingSimilar proteinsi
Cross-referencesi
Web resourcesi
NIEHS-SNPs |
Sequence databases
Select the link destinations: EMBLi GenBanki DDBJi Links Updated | AL136931 mRNA Translation: CAB66865.1 AM393631 mRNA Translation: CAL38507.1 EU567145 Genomic DNA Translation: ACB21048.1 AL359353 Genomic DNA No translation available. AL606726 Genomic DNA No translation available. CH471100 Genomic DNA Translation: EAW93293.1 CH471100 Genomic DNA Translation: EAW93294.1 BC011973 mRNA Translation: AAH11973.1 BC015829 mRNA Translation: AAH15829.2 BC016859 mRNA Translation: AAH16859.1 AK000612 mRNA Translation: BAA91287.1 Different initiation. |
CCDSi | CCDS1525.1 [Q969Z3-1] CCDS81425.1 [Q969Z3-2] |
RefSeqi | NP_001304267.1, NM_001317338.1 [Q969Z3-1] NP_001317971.1, NM_001331042.1 [Q969Z3-2] NP_060368.2, NM_017898.4 [Q969Z3-1] |
3D structure databases
AlphaFoldDBi | Q969Z3 |
SMRi | Q969Z3 |
ModBasei | Search... |
Protein-protein interaction databases
BioGRIDi | 120329, 36 interactors |
IntActi | Q969Z3, 10 interactors |
MINTi | Q969Z3 |
STRINGi | 9606.ENSP00000355880 |
Chemistry databases
ChEMBLi | CHEMBL4523423 |
PTM databases
iPTMneti | Q969Z3 |
PhosphoSitePlusi | Q969Z3 |
Genetic variation databases
BioMutai | MARC2 |
DMDMi | 74760692 |
Proteomic databases
EPDi | Q969Z3 |
jPOSTi | Q969Z3 |
MassIVEi | Q969Z3 |
MaxQBi | Q969Z3 |
PaxDbi | Q969Z3 |
PeptideAtlasi | Q969Z3 |
PRIDEi | Q969Z3 |
ProteomicsDBi | 75882 [Q969Z3-1] 75883 [Q969Z3-2] |
Protocols and materials databases
Antibodypediai | 2381, 138 antibodies from 21 providers |
DNASUi | 54996 |
Genome annotation databases
Ensembli | ENST00000359316.6; ENSP00000352266.2; ENSG00000117791.16 [Q969Z3-2] ENST00000366913.8; ENSP00000355880.3; ENSG00000117791.16 |
GeneIDi | 54996 |
KEGGi | hsa:54996 |
MANE-Selecti | ENST00000366913.8; ENSP00000355880.3; NM_017898.5; NP_060368.2 |
UCSCi | uc001hmq.4, human [Q969Z3-1] |
Organism-specific databases
CTDi | 54996 |
DisGeNETi | 54996 |
GeneCardsi | MTARC2 |
HGNCi | HGNC:26064, MTARC2 |
HPAi | ENSG00000117791, Tissue enhanced (kidney, liver) |
MIMi | 614127, gene |
neXtProti | NX_Q969Z3 |
OpenTargetsi | ENSG00000117791 |
PharmGKBi | PA142671345 |
VEuPathDBi | HostDB:ENSG00000117791 |
GenAtlasi | Search... |
Phylogenomic databases
eggNOGi | KOG2362, Eukaryota |
GeneTreei | ENSGT00940000159665 |
HOGENOMi | CLU_028286_6_2_1 |
InParanoidi | Q969Z3 |
OMAi | GYPFLLI |
OrthoDBi | 919343at2759 |
PhylomeDBi | Q969Z3 |
TreeFami | TF316807 |
Enzyme and pathway databases
BRENDAi | 1.7.2.1, 2681 |
PathwayCommonsi | Q969Z3 |
Reactomei | R-HSA-211945, Phase I - Functionalization of compounds |
SABIO-RKi | Q969Z3 |
SignaLinki | Q969Z3 |
Miscellaneous databases
BioGRID-ORCSi | 54996, 2 hits in 828 CRISPR screens |
ChiTaRSi | MARC2, human |
GeneWikii | MOSC2 |
GenomeRNAii | 54996 |
Pharosi | Q969Z3, Tbio |
PROi | PR:Q969Z3 |
RNActi | Q969Z3, protein |
SOURCEi | Search... |
Gene expression databases
Bgeei | ENSG00000117791, Expressed in left lobe of thyroid gland and 224 other tissues |
ExpressionAtlasi | Q969Z3, baseline and differential |
Genevisiblei | Q969Z3, HS |
Family and domain databases
InterProi | View protein in InterPro IPR005302, MoCF_Sase_C IPR005303, MOSC_N IPR011037, Pyrv_Knase-like_insert_dom_sf |
Pfami | View protein in Pfam PF03473, MOSC, 1 hit PF03476, MOSC_N, 1 hit |
SUPFAMi | SSF50800, SSF50800, 1 hit |
PROSITEi | View protein in PROSITE PS51340, MOSC, 1 hit |
MobiDBi | Search... |
Entry informationi
Entry namei | MARC2_HUMAN | |
Accessioni | Q969Z3Primary (citable) accession number: Q969Z3 Secondary accession number(s): B2D0R5 Q9NWU0 | |
Entry historyi | Integrated into UniProtKB/Swiss-Prot: | January 23, 2007 |
Last sequence update: | December 1, 2001 | |
Last modified: | May 25, 2022 | |
This is version 156 of the entry and version 1 of the sequence. See complete history. | ||
Entry statusi | Reviewed (UniProtKB/Swiss-Prot) | |
Annotation program | Chordata Protein Annotation Program | |
Disclaimer | Any medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care. |
Miscellaneousi
Keywords - Technical termi
Reference proteomeDocuments
- Human chromosome 1
Human chromosome 1: entries, gene names and cross-references to MIM - Human entries with genetic variants
List of human entries with genetic variants - Human variants curated from literature reports
Index of human variants curated from literature reports - MIM cross-references
Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot