UniProtKB - Q5VT66 (MARC1_HUMAN)
Protein
Mitochondrial amidoxime-reducing component 1
Gene
MTARC1
Organism
Homo sapiens (Human)
Status
Functioni
Catalyzes the reduction of N-oxygenated molecules, acting as a counterpart of cytochrome P450 and flavin-containing monooxygenases in metabolic cycles (PubMed:19053771, PubMed:21029045, PubMed:30397129). As a component of prodrug-converting system, reduces a multitude of N-hydroxylated prodrugs particularly amidoximes, leading to increased drug bioavailability (PubMed:19053771). 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, PubMed:19053771).3 Publications
Catalytic 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
Mo-molybdopterin3 PublicationsNote: Binds 1 Mo-molybdopterin (Mo-MPT) cofactor per subunit.2 Publications
Kineticsi
- KM=180 µM for benzamidoxime1 Publication
- KM=86 µM for NOHA1 Publication
- KM=272 µM for NHAM1 Publication
- Vmax=34 nmol/min/mg enzyme toward benzamidoxime1 Publication
- Vmax=105 nmol/min/mg enzyme toward benzamidoxime (at pH 6.0 and 37 degrees Celsius)1 Publication
- Vmax=55 nmol/min/mg enzyme toward NOHA1 Publication
- Vmax=43 nmol/min/mg enzyme toward NHAM1 Publication
Sites
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Metal bindingi | 273 | Molybdenum1 Publication | 1 |
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
- xenobiotic metabolic process Source: Reactome
Keywordsi
Molecular function | Oxidoreductase |
Ligand | Metal-binding, Molybdenum |
Enzyme and pathway databases
PathwayCommonsi | Q5VT66 |
Reactomei | R-HSA-211945, Phase I - Functionalization of compounds |
SIGNORi | Q5VT66 |
Names & Taxonomyi
Protein namesi | Recommended name: Mitochondrial amidoxime-reducing component 11 Publication (EC:1.7.-.-1 Publication)Short name: mARC11 Publication Alternative name(s): Molybdenum cofactor sulfurase C-terminal domain-containing protein 11 Publication Short name: MOSC domain-containing protein 1 Short name: Moco sulfurase C-terminal domain-containing protein 1 |
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:26189, MTARC1 |
MIMi | 614126, gene |
neXtProti | NX_Q5VT66 |
VEuPathDBi | HostDB:ENSG00000186205.12 |
Subcellular locationi
Mitochondrion
- Mitochondrion outer membrane 1 Publication; Single-pass type II membrane protein 1 Publication
Other locations
- Membrane Curated; Lipid-anchor Curated
Note: Mitochondrial import is mediated by AA 1-40 and requires ATP.
Mitochondrion
- mitochondrial outer membrane Source: Reactome
- mitochondrion Source: HPA
Other locations
- integral component of membrane Source: UniProtKB-KW
- nitric-oxide synthase complex Source: FlyBase
Topology
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Topological domaini | 2 – 20 | Mitochondrial matrix1 PublicationAdd BLAST | 19 | |
Transmembranei | 21 – 40 | Helical; Signal-anchor for type II membrane proteinSequence analysisAdd BLAST | 20 | |
Topological domaini | 41 – 337 | Cytoplasmic1 PublicationAdd BLAST | 297 |
Keywords - Cellular componenti
Membrane, Mitochondrion, Mitochondrion outer membranePathology & Biotechi
Organism-specific databases
DisGeNETi | 64757 |
OpenTargetsi | ENSG00000186205 |
PharmGKBi | PA142671344 |
Miscellaneous databases
Pharosi | Q5VT66, Tbio |
Chemistry databases
ChEMBLi | CHEMBL3706559 |
Genetic variation databases
BioMutai | MARC1 |
DMDMi | 74746896 |
PTM / Processingi
Molecule processing
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Initiator methioninei | Removed2 Publications | |||
ChainiPRO_0000273335 | 2 – 337 | Mitochondrial amidoxime-reducing component 1Add BLAST | 336 |
Amino acid modifications
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Lipidationi | 2 | N-myristoyl glycine2 Publications | 1 |
Keywords - PTMi
Lipoprotein, MyristateProteomic databases
EPDi | Q5VT66 |
jPOSTi | Q5VT66 |
MassIVEi | Q5VT66 |
MaxQBi | Q5VT66 |
PaxDbi | Q5VT66 |
PeptideAtlasi | Q5VT66 |
PRIDEi | Q5VT66 |
ProteomicsDBi | 65307 [Q5VT66-1] 65308 [Q5VT66-2] 65309 [Q5VT66-3] |
PTM databases
iPTMneti | Q5VT66 |
PhosphoSitePlusi | Q5VT66 |
SwissPalmi | Q5VT66 |
Expressioni
Gene expression databases
Bgeei | ENSG00000186205, Expressed in adipose tissue and 202 other tissues |
ExpressionAtlasi | Q5VT66, baseline and differential |
Genevisiblei | Q5VT66, HS |
Organism-specific databases
HPAi | ENSG00000186205, Tissue enhanced (adipose tissue, breast) |
Interactioni
Subunit structurei
Component of a complex composed of cytochrome b5, NADH-cytochrome b5 reductase and MTARC1.
Binary interactionsi
Hide detailsQ5VT66
With | #Exp. | IntAct |
---|---|---|
EPHB6 - isoform 2 [O15197-2] | 3 | EBI-11903927,EBI-10182490 |
NAA11 [Q9BSU3] | 3 | EBI-11903927,EBI-2585120 |
Protein-protein interaction databases
BioGRIDi | 122271, 45 interactors |
IntActi | Q5VT66, 15 interactors |
STRINGi | 9606.ENSP00000355877 |
Miscellaneous databases
RNActi | Q5VT66, protein |
Structurei
Secondary structure
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details3D structure databases
SMRi | Q5VT66 |
ModBasei | Search... |
PDBe-KBi | Search... |
Family & Domainsi
Domains and Repeats
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Domaini | 187 – 335 | MOSCPROSITE-ProRule annotationAdd BLAST | 149 |
Region
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Regioni | 52 – 92 | Molybdopterin-binding1 PublicationAdd BLAST | 41 | |
Regioni | 93 – 183 | MOSC N-terminal region1 PublicationAdd BLAST | 91 | |
Regioni | 210 – 335 | Molybdopterin-binding1 PublicationAdd BLAST | 126 |
Domaini
Comprises two structural domains, the molybdenum cofactor/Moco sulfurase C-terminal (MOSC) domain and the MOSC N-terminal region, forming a cleft that accommodates Moco. The MOSC domain, which contains a large seven-stranded mostly antiparallel beta-barrel, engages multiple interactions with Moco both pterin ring and phosphate group, allowing for a tight coordination of Moco within the core of the enzyme.1 Publication
Keywords - Domaini
Signal-anchor, Transmembrane, Transmembrane helixPhylogenomic databases
eggNOGi | KOG2362, Eukaryota |
GeneTreei | ENSGT00940000162410 |
HOGENOMi | CLU_028286_6_0_1 |
InParanoidi | Q5VT66 |
OMAi | YPVKGCA |
OrthoDBi | 919343at2759 |
PhylomeDBi | Q5VT66 |
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 (3+)i Sequence
Sequence statusi: Complete.
: The displayed sequence is further processed into a mature form. Sequence processingi
This entry describes 3 produced by isoformsialternative splicing. AlignAdd to basketThis entry has 3 described isoforms and 3 potential isoforms that are computationally mapped.Show allAlign All
Isoform 1 (identifier: Q5VT66-1) [UniParc]FASTAAdd to basket
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
MGAAGSSALA RFVLLAQSRP GWLGVAALGL TAVALGAVAW RRAWPTRRRR
60 70 80 90 100
LLQQVGTVAQ LWIYPVKSCK GVPVSEAECT AMGLRSGNLR DRFWLVINQE
110 120 130 140 150
GNMVTARQEP RLVLISLTCD GDTLTLSAAY TKDLLLPIKT PTTNAVHKCR
160 170 180 190 200
VHGLEIEGRD CGEATAQWIT SFLKSQPYRL VHFEPHMRPR RPHQIADLFR
210 220 230 240 250
PKDQIAYSDT SPFLILSEAS LADLNSRLEK KVKATNFRPN IVISGCDVYA
260 270 280 290 300
EDSWDELLIG DVELKRVMAC SRCILTTVDP DTGVMSRKEP LETLKSYRQC
310 320 330
DPSERKLYGK SPLFGQYFVL ENPGTIKVGD PVYLLGQ
Computationally mapped potential isoform sequencesi
There are 3 potential isoforms mapped to this entry.BLASTAlignShow allAdd to basketH7BYZ9 | H7BYZ9_HUMAN | Mitochondrial amidoxime-reducing co... | MTARC1 | 263 | Annotation score: | ||
X6RIL1 | X6RIL1_HUMAN | Mitochondrial amidoxime-reducing co... | MTARC1 | 119 | Annotation score: | ||
H0YDX4 | H0YDX4_HUMAN | Mitochondrial amidoxime-reducing co... | MTARC1 | 99 | Annotation score: |
Sequence cautioni
The sequence BAB15333 differs from that shown. Reason: Frameshift.Curated
Natural variant
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Natural variantiVAR_062273 | 15 | L → H1 PublicationCorresponds to variant dbSNP:rs72470572Ensembl. | 1 | |
Natural variantiVAR_056941 | 96 | V → L No effect on binding of the molybdenum cofactor; no significant effect on catalytic efficiency toward benzamidoxime; no significant effect on affinity for benzamidoxime. 2 PublicationsCorresponds to variant dbSNP:rs12023067EnsemblClinVar. | 1 | |
Natural variantiVAR_030129 | 165 | T → A No effect on binding of the molybdenum cofactor; no significant effect on catalytic efficiency toward benzamidoxime; no significant effect on affinity for benzamidoxime. 5 PublicationsCorresponds to variant dbSNP:rs2642438Ensembl. | 1 | |
Natural variantiVAR_030130 | 187 | M → K No effect on binding of the molybdenum cofactor; no significant effect on catalytic efficiency toward benzamidoxime; no significant effect on affinity for benzamidoxime. 2 PublicationsCorresponds to variant dbSNP:rs17850677EnsemblClinVar. | 1 | |
Natural variantiVAR_030131 | 246 | C → S No effect on binding of the molybdenum cofactor; no significant effect on catalytic efficiency toward benzamidoxime; no significant effect on affinity for benzamidoxime. 2 PublicationsCorresponds to variant dbSNP:rs3738178Ensembl. | 1 | |
Natural variantiVAR_062274 | 247 | D → H No effect on binding of the molybdenum cofactor; no significant effect on catalytic efficiency toward benzamidoxime; no significant effect on affinity for benzamidoxime. 2 PublicationsCorresponds to variant dbSNP:rs72470601Ensembl. | 1 | |
Natural variantiVAR_030132 | 268 | M → I No effect on binding of the molybdenum cofactor; no significant effect on catalytic efficiency toward benzamidoxime; no significant effect on affinity for benzamidoxime. 1 PublicationCorresponds to variant dbSNP:rs2642419Ensembl. | 1 |
Alternative sequence
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Alternative sequenceiVSP_022511 | 1 – 102 | Missing in isoform 3. 1 PublicationAdd BLAST | 102 | |
Alternative sequenceiVSP_022512 | 251 | E → EVTLCPFGSFLGFDFFFK in isoform 2 and isoform 3. 1 Publication | 1 |
Sequence databases
Select the link destinations: EMBLi GenBanki DDBJi Links Updated | AK026043 mRNA Translation: BAB15333.1 Frameshift. AK094105 mRNA Translation: BAC04286.1 AK290812 mRNA Translation: BAF83501.1 EU563849 Genomic DNA Translation: ACB21046.1 AL445423 Genomic DNA No translation available. AL606726 Genomic DNA No translation available. CH471100 Genomic DNA Translation: EAW93291.1 BC010619 mRNA Translation: AAH10619.1 |
CCDSi | CCDS1526.1 [Q5VT66-1] |
RefSeqi | NP_073583.3, NM_022746.3 [Q5VT66-1] XP_011508202.1, XM_011509900.2 [Q5VT66-2] XP_011508206.1, XM_011509904.2 [Q5VT66-3] |
Genome annotation databases
Ensembli | ENST00000366910; ENSP00000355877; ENSG00000186205 [Q5VT66-1] |
GeneIDi | 64757 |
KEGGi | hsa:64757 |
UCSCi | uc001hms.4, human [Q5VT66-1] |
Keywords - Coding sequence diversityi
Alternative splicingSimilar proteinsi
Cross-referencesi
Web resourcesi
NIEHS-SNPs |
Sequence databases
Select the link destinations: EMBLi GenBanki DDBJi Links Updated | AK026043 mRNA Translation: BAB15333.1 Frameshift. AK094105 mRNA Translation: BAC04286.1 AK290812 mRNA Translation: BAF83501.1 EU563849 Genomic DNA Translation: ACB21046.1 AL445423 Genomic DNA No translation available. AL606726 Genomic DNA No translation available. CH471100 Genomic DNA Translation: EAW93291.1 BC010619 mRNA Translation: AAH10619.1 |
CCDSi | CCDS1526.1 [Q5VT66-1] |
RefSeqi | NP_073583.3, NM_022746.3 [Q5VT66-1] XP_011508202.1, XM_011509900.2 [Q5VT66-2] XP_011508206.1, XM_011509904.2 [Q5VT66-3] |
3D structure databases
Select the link destinations: PDBei RCSB PDBi PDBji Links Updated | PDB entry | Method | Resolution (Å) | Chain | Positions | PDBsum |
6FW2 | X-ray | 1.78 | A | 53-337 | [»] | |
SMRi | Q5VT66 | |||||
ModBasei | Search... | |||||
PDBe-KBi | Search... |
Protein-protein interaction databases
BioGRIDi | 122271, 45 interactors |
IntActi | Q5VT66, 15 interactors |
STRINGi | 9606.ENSP00000355877 |
Chemistry databases
ChEMBLi | CHEMBL3706559 |
PTM databases
iPTMneti | Q5VT66 |
PhosphoSitePlusi | Q5VT66 |
SwissPalmi | Q5VT66 |
Genetic variation databases
BioMutai | MARC1 |
DMDMi | 74746896 |
Proteomic databases
EPDi | Q5VT66 |
jPOSTi | Q5VT66 |
MassIVEi | Q5VT66 |
MaxQBi | Q5VT66 |
PaxDbi | Q5VT66 |
PeptideAtlasi | Q5VT66 |
PRIDEi | Q5VT66 |
ProteomicsDBi | 65307 [Q5VT66-1] 65308 [Q5VT66-2] 65309 [Q5VT66-3] |
Protocols and materials databases
Antibodypediai | 34627, 116 antibodies |
Genome annotation databases
Ensembli | ENST00000366910; ENSP00000355877; ENSG00000186205 [Q5VT66-1] |
GeneIDi | 64757 |
KEGGi | hsa:64757 |
UCSCi | uc001hms.4, human [Q5VT66-1] |
Organism-specific databases
CTDi | 64757 |
DisGeNETi | 64757 |
GeneCardsi | MTARC1 |
HGNCi | HGNC:26189, MTARC1 |
HPAi | ENSG00000186205, Tissue enhanced (adipose tissue, breast) |
MIMi | 614126, gene |
neXtProti | NX_Q5VT66 |
OpenTargetsi | ENSG00000186205 |
PharmGKBi | PA142671344 |
VEuPathDBi | HostDB:ENSG00000186205.12 |
GenAtlasi | Search... |
Phylogenomic databases
eggNOGi | KOG2362, Eukaryota |
GeneTreei | ENSGT00940000162410 |
HOGENOMi | CLU_028286_6_0_1 |
InParanoidi | Q5VT66 |
OMAi | YPVKGCA |
OrthoDBi | 919343at2759 |
PhylomeDBi | Q5VT66 |
TreeFami | TF316807 |
Enzyme and pathway databases
PathwayCommonsi | Q5VT66 |
Reactomei | R-HSA-211945, Phase I - Functionalization of compounds |
SIGNORi | Q5VT66 |
Miscellaneous databases
BioGRID-ORCSi | 64757, 3 hits in 766 CRISPR screens |
ChiTaRSi | MARC1, human |
GenomeRNAii | 64757 |
Pharosi | Q5VT66, Tbio |
PROi | PR:Q5VT66 |
RNActi | Q5VT66, protein |
SOURCEi | Search... |
Gene expression databases
Bgeei | ENSG00000186205, Expressed in adipose tissue and 202 other tissues |
ExpressionAtlasi | Q5VT66, baseline and differential |
Genevisiblei | Q5VT66, 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 |
ProtoNeti | Search... |
MobiDBi | Search... |
Entry informationi
Entry namei | MARC1_HUMAN | |
Accessioni | Q5VT66Primary (citable) accession number: Q5VT66 Secondary accession number(s): A8K447 Q9H6C7 | |
Entry historyi | Integrated into UniProtKB/Swiss-Prot: | January 23, 2007 |
Last sequence update: | December 7, 2004 | |
Last modified: | April 7, 2021 | |
This is version 138 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
3D-structure, 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 - PDB cross-references
Index of Protein Data Bank (PDB) cross-references