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Protein

Mitochondrial amidoxime-reducing component 1

Gene

MARC1

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-Experimental evidence at protein leveli

Functioni

As a component of an N-hydroxylated prodrug-converting complex required to reduce N-hydroxylated prodrugs, such as benzamidoxime. Also able to reduce N(omega)-hydroxy-L-arginine (NOHA) and N(omega)-hydroxy-N(delta)-methyl-L-arginine (NHAM) into L-arginine and N(delta)-methyl-L-arginine, respectively.1 Publication

Cofactori

Mo-molybdopterin2 PublicationsNote: Binds 1 Mo-molybdopterin (Mo-MPT) cofactor per subunit.2 Publications

Kineticsi

  1. KM=180 µM for benzamidoxime2 Publications
  2. KM=86 µM for NOHA2 Publications
  3. KM=272 µM for NHAM2 Publications
  1. Vmax=34 nmol/min/mg enzyme toward benzamidoxime2 Publications
  2. Vmax=105 nmol/min/mg enzyme toward benzamidoxime (PubMed:24423752, at pH 6.0 and 37 degrees Celsius)2 Publications
  3. Vmax=55 nmol/min/mg enzyme toward NOHA2 Publications
  4. Vmax=43 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
  • pyridoxal phosphate binding Source: InterPro

GO - Biological processi

  • detoxification of nitrogen compound Source: UniProtKB
  • nitrate metabolic process Source: UniProtKB
  • oxidation-reduction process Source: UniProtKB
Complete GO annotation...

Keywords - Molecular functioni

Oxidoreductase

Keywords - Ligandi

Molybdenum

Names & Taxonomyi

Protein namesi
Recommended name:
Mitochondrial amidoxime-reducing component 1 (EC:1.-.-.-)
Short name:
mARC1
Alternative name(s):
Molybdenum cofactor sulfurase C-terminal domain-containing protein 1
Short name:
MOSC domain-containing protein 1
Short name:
Moco sulfurase C-terminal domain-containing protein 1
Gene namesi
Name:MARC1
Synonyms:MOSC1
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 1

Organism-specific databases

HGNCiHGNC:26189. MARC1.

Subcellular locationi

Topology

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Topological domaini2 – 20Mitochondrial matrix1 PublicationAdd BLAST19
Transmembranei21 – 40Helical; Signal-anchor for type II membrane proteinSequence analysisAdd BLAST20
Topological domaini41 – 337Cytoplasmic1 PublicationAdd BLAST297

GO - Cellular componenti

  • integral component of membrane Source: UniProtKB-KW
  • mitochondrial inner membrane Source: Ensembl
  • mitochondrial outer membrane Source: UniProtKB-SubCell
  • mitochondrion Source: UniProtKB
Complete GO annotation...

Keywords - Cellular componenti

Membrane, Mitochondrion, Mitochondrion outer membrane

Pathology & Biotechi

Organism-specific databases

DisGeNETi64757.
OpenTargetsiENSG00000186205.
PharmGKBiPA142671344.

Chemistry databases

ChEMBLiCHEMBL3706559.

Polymorphism and mutation databases

BioMutaiMARC1.
DMDMi74746896.

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Initiator methionineiRemoved2 Publications
ChainiPRO_00002733352 – 337Mitochondrial amidoxime-reducing component 1Add BLAST336

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Lipidationi2N-myristoyl glycine2 Publications1

Keywords - PTMi

Lipoprotein, Myristate

Proteomic databases

EPDiQ5VT66.
MaxQBiQ5VT66.
PaxDbiQ5VT66.
PeptideAtlasiQ5VT66.
PRIDEiQ5VT66.

PTM databases

iPTMnetiQ5VT66.
PhosphoSitePlusiQ5VT66.
SwissPalmiQ5VT66.

Expressioni

Gene expression databases

BgeeiENSG00000186205.
CleanExiHS_MOSC1.
ExpressionAtlasiQ5VT66. baseline and differential.
GenevisibleiQ5VT66. HS.

Organism-specific databases

HPAiHPA028702.

Interactioni

Subunit structurei

Component of a complex composed of cytochrome b5, NADH-cytochrome b5 reductase and MARC1.

Protein-protein interaction databases

BioGridi122271. 20 interactors.
STRINGi9606.ENSP00000355877.

Structurei

3D structure databases

ProteinModelPortaliQ5VT66.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Domaini187 – 335MOSCPROSITE-ProRule annotationAdd BLAST149

Sequence similaritiesi

Contains 1 MOSC domain.PROSITE-ProRule annotation

Keywords - Domaini

Signal-anchor, Transmembrane, Transmembrane helix

Phylogenomic databases

eggNOGiKOG2362. Eukaryota.
COG3217. LUCA.
GeneTreeiENSGT00530000063150.
HOVERGENiHBG081982.
InParanoidiQ5VT66.
OMAiCDPSERK.
OrthoDBiEOG091G0S9F.
PhylomeDBiQ5VT66.
TreeFamiTF316807.

Family and domain databases

InterProiIPR005302. MoCF_Sase_C.
IPR005303. MOSC_N.
IPR011037. Pyrv_Knase-like_insert_dom.
[Graphical view]
PfamiPF03473. MOSC. 1 hit.
PF03476. MOSC_N. 1 hit.
[Graphical view]
SUPFAMiSSF50800. SSF50800. 2 hits.
PROSITEiPS51340. MOSC. 1 hit.
[Graphical view]

Sequences (3)i

Sequence statusi: Complete.

Sequence processingi: The displayed sequence is further processed into a mature form.

This entry describes 3 isoformsi produced by alternative splicing. AlignAdd to basket

Isoform 1 (identifier: Q5VT66-1) [UniParc]FASTAAdd to basket

This isoform has been chosen as the 'canonical' sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry.

« Hide

        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
Length:337
Mass (Da):37,499
Last modified:December 7, 2004 - v1
Checksum:iF983AB08F3D646C4
GO
Isoform 2 (identifier: Q5VT66-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     251-251: E → EVTLCPFGSFLGFDFFFK

Note: No experimental confirmation available.
Show »
Length:354
Mass (Da):39,454
Checksum:iCC368AD4B543AD51
GO
Isoform 3 (identifier: Q5VT66-3) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     1-102: Missing.
     251-251: E → EVTLCPFGSFLGFDFFFK

Note: No experimental confirmation available.
Show »
Length:252
Mass (Da):28,439
Checksum:i0FEF88317AF6C9D2
GO

Sequence cautioni

The sequence BAB15333 differs from that shown. Reason: Frameshift at position 139.Curated
The sequence CAH72118 differs from that shown. Reason: Erroneous gene model prediction.Curated

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_06227315L → H.1 PublicationCorresponds to variant rs72470572dbSNPEnsembl.1
Natural variantiVAR_05694196V → L Common polymorphism in Caucasian population; 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 rs12023067dbSNPEnsembl.1
Natural variantiVAR_030129165T → 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 rs2642438dbSNPEnsembl.1
Natural variantiVAR_030130187M → 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 rs17850677dbSNPEnsembl.1
Natural variantiVAR_030131246C → 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 rs3738178dbSNPEnsembl.1
Natural variantiVAR_062274247D → 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 rs72470601dbSNPEnsembl.1
Natural variantiVAR_030132268M → 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 rs2642419dbSNPEnsembl.1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_0225111 – 102Missing in isoform 3. 1 PublicationAdd BLAST102
Alternative sequenceiVSP_022512251E → EVTLCPFGSFLGFDFFFK in isoform 2 and isoform 3. 1 Publication1

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.
AL606726, AL445423 Genomic DNA. Translation: CAH71881.1.
AL445423 Genomic DNA. Translation: CAH72116.1.
AL445423 Genomic DNA. Translation: CAH72118.1. Sequence problems.
AL445423, AL606726 Genomic DNA. Translation: CAH72119.1.
CH471100 Genomic DNA. Translation: EAW93291.1.
BC010619 mRNA. Translation: AAH10619.1.
CCDSiCCDS1526.1. [Q5VT66-1]
RefSeqiNP_073583.3. NM_022746.3. [Q5VT66-1]
XP_011508202.1. XM_011509900.2. [Q5VT66-2]
XP_011508206.1. XM_011509904.2. [Q5VT66-3]
UniGeneiHs.497816.

Genome annotation databases

EnsembliENST00000366910; ENSP00000355877; ENSG00000186205. [Q5VT66-1]
GeneIDi64757.
KEGGihsa:64757.
UCSCiuc001hms.4. human. [Q5VT66-1]

Keywords - Coding sequence diversityi

Alternative splicing, Polymorphism

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.
AL606726, AL445423 Genomic DNA. Translation: CAH71881.1.
AL445423 Genomic DNA. Translation: CAH72116.1.
AL445423 Genomic DNA. Translation: CAH72118.1. Sequence problems.
AL445423, AL606726 Genomic DNA. Translation: CAH72119.1.
CH471100 Genomic DNA. Translation: EAW93291.1.
BC010619 mRNA. Translation: AAH10619.1.
CCDSiCCDS1526.1. [Q5VT66-1]
RefSeqiNP_073583.3. NM_022746.3. [Q5VT66-1]
XP_011508202.1. XM_011509900.2. [Q5VT66-2]
XP_011508206.1. XM_011509904.2. [Q5VT66-3]
UniGeneiHs.497816.

3D structure databases

ProteinModelPortaliQ5VT66.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi122271. 20 interactors.
STRINGi9606.ENSP00000355877.

Chemistry databases

ChEMBLiCHEMBL3706559.

PTM databases

iPTMnetiQ5VT66.
PhosphoSitePlusiQ5VT66.
SwissPalmiQ5VT66.

Polymorphism and mutation databases

BioMutaiMARC1.
DMDMi74746896.

Proteomic databases

EPDiQ5VT66.
MaxQBiQ5VT66.
PaxDbiQ5VT66.
PeptideAtlasiQ5VT66.
PRIDEiQ5VT66.

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000366910; ENSP00000355877; ENSG00000186205. [Q5VT66-1]
GeneIDi64757.
KEGGihsa:64757.
UCSCiuc001hms.4. human. [Q5VT66-1]

Organism-specific databases

CTDi64757.
DisGeNETi64757.
GeneCardsiMARC1.
H-InvDBHIX0001608.
HGNCiHGNC:26189. MARC1.
HPAiHPA028702.
MIMi614126. gene.
neXtProtiNX_Q5VT66.
OpenTargetsiENSG00000186205.
PharmGKBiPA142671344.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG2362. Eukaryota.
COG3217. LUCA.
GeneTreeiENSGT00530000063150.
HOVERGENiHBG081982.
InParanoidiQ5VT66.
OMAiCDPSERK.
OrthoDBiEOG091G0S9F.
PhylomeDBiQ5VT66.
TreeFamiTF316807.

Miscellaneous databases

GenomeRNAii64757.
PROiQ5VT66.
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000186205.
CleanExiHS_MOSC1.
ExpressionAtlasiQ5VT66. baseline and differential.
GenevisibleiQ5VT66. HS.

Family and domain databases

InterProiIPR005302. MoCF_Sase_C.
IPR005303. MOSC_N.
IPR011037. Pyrv_Knase-like_insert_dom.
[Graphical view]
PfamiPF03473. MOSC. 1 hit.
PF03476. MOSC_N. 1 hit.
[Graphical view]
SUPFAMiSSF50800. SSF50800. 2 hits.
PROSITEiPS51340. MOSC. 1 hit.
[Graphical view]
ProtoNetiSearch...

Entry informationi

Entry nameiMARC1_HUMAN
AccessioniPrimary (citable) accession number: Q5VT66
Secondary accession number(s): A8K447
, B2D078, Q5VVS9, Q5VVT0, Q5VVT1, Q8N9P5, Q96FN8, Q9H6C7
Entry historyi
Integrated into UniProtKB/Swiss-Prot: January 23, 2007
Last sequence update: December 7, 2004
Last modified: November 30, 2016
This is version 105 of the entry and version 1 of the sequence. [Complete history]
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program
DisclaimerAny 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

Complete proteome, Reference proteome

Documents

  1. Human chromosome 1
    Human chromosome 1: entries, gene names and cross-references to MIM
  2. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  3. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  4. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  5. SIMILARITY comments
    Index of protein domains and families

Similar proteinsi

Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into one UniRef entry.
90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.