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Protein

Mitochondrial amidoxime reducing component 2

Gene

MARC2

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

Functioni

As a component of the benzamidoxime 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-molybdopterin1 PublicationNote: Binds 1 Mo-molybdopterin (Mo-MPT) cofactor per subunit.1 Publication

Kineticsi

  1. KM=830 µM for benzamidoxime2 Publications
  2. KM=400 µM for benzamidoxime (at pH 6.0 and 37 degrees Celsius)1 Publication
  3. KM=3 mM for NOHA2 Publications
  4. KM=3.7 mM for NHAM2 Publications
  1. Vmax=307 nmol/min/mg enzyme toward benzamidoxime2 Publications
  2. Vmax=119 nmol/min/mg enzyme toward benzamidoxime (at pH 6.0 and 37 degrees Celsius)1 Publication
  3. Vmax=373 nmol/min/mg enzyme toward NOHA2 Publications
  4. Vmax=36.5 nmol/min/mg enzyme toward NHAM2 Publications

GO - Molecular functioni

GO - Biological processi

Keywordsi

Molecular functionOxidoreductase
LigandMolybdenum

Enzyme and pathway databases

ReactomeiR-HSA-211945 Phase I - Functionalization of compounds

Names & Taxonomyi

Protein namesi
Recommended name:
Mitochondrial amidoxime reducing component 2 (EC:1.-.-.-)
Short name:
mARC2
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
Name:MARC2
Synonyms:MOSC2
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 1

Organism-specific databases

EuPathDBiHostDB:ENSG00000117791.15
HGNCiHGNC:26064 MARC2
MIMi614127 gene
neXtProtiNX_Q969Z3

Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

Keywords - Cellular componenti

Membrane, Mitochondrion, Mitochondrion outer membrane, Peroxisome

Pathology & Biotechi

Organism-specific databases

DisGeNETi54996
OpenTargetsiENSG00000117791
PharmGKBiPA142671345

Polymorphism and mutation databases

BioMutaiMARC2
DMDMi74760692

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transit peptidei1 – 35MitochondrionSequence analysisAdd BLAST35
ChainiPRO_000027334036 – 335Mitochondrial amidoxime reducing component 2Add BLAST300

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Cross-linki59Glycyl lysine isopeptide (Lys-Gly) (interchain with G-Cter in ubiquitin)1 Publication
Cross-linki138Glycyl lysine isopeptide (Lys-Gly) (interchain with G-Cter in ubiquitin)1 Publication
Cross-linki144Glycyl lysine isopeptide (Lys-Gly) (interchain with G-Cter in ubiquitin)1 Publication
Modified residuei156N6-acetyllysine; alternateBy similarity1
Cross-linki156Glycyl lysine isopeptide (Lys-Gly) (interchain with G-Cter in ubiquitin); alternate1 Publication
Cross-linki166Glycyl lysine isopeptide (Lys-Gly) (interchain with G-Cter in ubiquitin)1 Publication
Cross-linki173Glycyl lysine isopeptide (Lys-Gly) (interchain with G-Cter in ubiquitin)1 Publication
Cross-linki187Glycyl lysine isopeptide (Lys-Gly) (interchain with G-Cter in ubiquitin)1 Publication
Cross-linki287Glycyl lysine isopeptide (Lys-Gly) (interchain with G-Cter in ubiquitin)1 Publication
Cross-linki294Glycyl lysine isopeptide (Lys-Gly) (interchain with G-Cter in ubiquitin)1 Publication

Post-translational modificationi

Ubiquitinated by PRKN during mitophagy, leading to its degradation and enhancement of mitophagy. Deubiquitinated by USP30.1 Publication

Keywords - PTMi

Acetylation, Isopeptide bond, Ubl conjugation

Proteomic databases

EPDiQ969Z3
MaxQBiQ969Z3
PaxDbiQ969Z3
PeptideAtlasiQ969Z3
PRIDEiQ969Z3
ProteomicsDBi75882
75883 [Q969Z3-2]

PTM databases

iPTMnetiQ969Z3
PhosphoSitePlusiQ969Z3

Expressioni

Gene expression databases

BgeeiENSG00000117791 Expressed in 212 organ(s), highest expression level in left lobe of thyroid gland
CleanExiHS_MOSC2
ExpressionAtlasiQ969Z3 baseline and differential
GenevisibleiQ969Z3 HS

Organism-specific databases

HPAiHPA015085
HPA017572

Interactioni

Subunit structurei

Component of a complex composed of cytochrome b5, NADH-cytochrome b5 reductase (CYB5R3) and MARC2.By similarity

Protein-protein interaction databases

BioGridi120329, 5 interactors
IntActiQ969Z3, 4 interactors
STRINGi9606.ENSP00000355880

Structurei

3D structure databases

ProteinModelPortaliQ969Z3
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Domaini188 – 334MOSCPROSITE-ProRule annotationAdd BLAST147

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiKOG2362 Eukaryota
COG3217 LUCA
GeneTreeiENSGT00530000063150
HOVERGENiHBG081982
InParanoidiQ969Z3
OMAiARQYPQM
OrthoDBiEOG091G0S9F
PhylomeDBiQ969Z3
TreeFamiTF316807

Family and domain databases

InterProiView protein in InterPro
IPR005302 MoCF_Sase_C
IPR005303 MOSC_N
IPR011037 Pyrv_Knase-like_insert_dom_sf
PfamiView protein in Pfam
PF03473 MOSC, 1 hit
PF03476 MOSC_N, 1 hit
SUPFAMiSSF50800 SSF50800, 2 hits
PROSITEiView protein in PROSITE
PS51340 MOSC, 1 hit

Sequences (2+)i

Sequence statusi: Complete.

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

This entry describes 2 isoformsi produced by alternative splicing. AlignAdd to basket

This entry has 2 described isoforms and 2 potential isoforms that are computationally mapped.Show allAlign All

Isoform 1 (identifier: Q969Z3-1) [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
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
Length:335
Mass (Da):38,023
Last modified:December 1, 2001 - v1
Checksum:i4C6A9B8B9AC0F74D
GO
Isoform 2 (identifier: Q969Z3-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     251-257: DTWDELL → ASATRRD
     258-335: Missing.

Note: No experimental confirmation available.
Show »
Length:257
Mass (Da):29,103
Checksum:i8938E1231A0ACB9B
GO

Computationally mapped potential isoform sequencesi

There are 2 potential isoforms mapped to this entry.BLASTAlignShow allAdd to basket
EntryEntry nameProtein names
Gene namesLengthAnnotation
X1WI34X1WI34_HUMAN
Mitochondrial amidoxime reducing co...
MARC2
172Annotation score:
F6V6Z1F6V6Z1_HUMAN
Mitochondrial amidoxime reducing co...
MARC2
94Annotation score:

Sequence cautioni

The sequence BAA91287 differs from that shown. Reason: Erroneous initiation. Translation N-terminally extended.Curated

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti169T → A in CAL38507 (PubMed:17974005).Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_0622753A → S1 PublicationCorresponds to variant dbSNP:rs72472370Ensembl.1
Natural variantiVAR_030133244G → 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_070777245C → 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 keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_022513251 – 257DTWDELL → ASATRRD in isoform 2. 1 Publication7
Alternative sequenceiVSP_022514258 – 335Missing in isoform 2. 1 PublicationAdd BLAST78

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.
CCDSiCCDS1525.1 [Q969Z3-1]
CCDS81425.1 [Q969Z3-2]
RefSeqiNP_001304267.1, NM_001317338.1 [Q969Z3-1]
NP_001317971.1, NM_001331042.1 [Q969Z3-2]
NP_060368.2, NM_017898.4 [Q969Z3-1]
UniGeneiHs.369042

Genome annotation databases

EnsembliENST00000359316; ENSP00000352266; ENSG00000117791 [Q969Z3-2]
ENST00000366913; ENSP00000355880; ENSG00000117791 [Q969Z3-1]
GeneIDi54996
KEGGihsa:54996
UCSCiuc001hmq.4 human [Q969Z3-1]

Keywords - Coding sequence diversityi

Alternative splicing, Polymorphism

Similar 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.
CCDSiCCDS1525.1 [Q969Z3-1]
CCDS81425.1 [Q969Z3-2]
RefSeqiNP_001304267.1, NM_001317338.1 [Q969Z3-1]
NP_001317971.1, NM_001331042.1 [Q969Z3-2]
NP_060368.2, NM_017898.4 [Q969Z3-1]
UniGeneiHs.369042

3D structure databases

ProteinModelPortaliQ969Z3
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi120329, 5 interactors
IntActiQ969Z3, 4 interactors
STRINGi9606.ENSP00000355880

PTM databases

iPTMnetiQ969Z3
PhosphoSitePlusiQ969Z3

Polymorphism and mutation databases

BioMutaiMARC2
DMDMi74760692

Proteomic databases

EPDiQ969Z3
MaxQBiQ969Z3
PaxDbiQ969Z3
PeptideAtlasiQ969Z3
PRIDEiQ969Z3
ProteomicsDBi75882
75883 [Q969Z3-2]

Protocols and materials databases

DNASUi54996
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000359316; ENSP00000352266; ENSG00000117791 [Q969Z3-2]
ENST00000366913; ENSP00000355880; ENSG00000117791 [Q969Z3-1]
GeneIDi54996
KEGGihsa:54996
UCSCiuc001hmq.4 human [Q969Z3-1]

Organism-specific databases

CTDi54996
DisGeNETi54996
EuPathDBiHostDB:ENSG00000117791.15
GeneCardsiMARC2
HGNCiHGNC:26064 MARC2
HPAiHPA015085
HPA017572
MIMi614127 gene
neXtProtiNX_Q969Z3
OpenTargetsiENSG00000117791
PharmGKBiPA142671345
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG2362 Eukaryota
COG3217 LUCA
GeneTreeiENSGT00530000063150
HOVERGENiHBG081982
InParanoidiQ969Z3
OMAiARQYPQM
OrthoDBiEOG091G0S9F
PhylomeDBiQ969Z3
TreeFamiTF316807

Enzyme and pathway databases

ReactomeiR-HSA-211945 Phase I - Functionalization of compounds

Miscellaneous databases

ChiTaRSiMARC2 human
GeneWikiiMOSC2
GenomeRNAii54996
PROiPR:Q969Z3
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000117791 Expressed in 212 organ(s), highest expression level in left lobe of thyroid gland
CleanExiHS_MOSC2
ExpressionAtlasiQ969Z3 baseline and differential
GenevisibleiQ969Z3 HS

Family and domain databases

InterProiView protein in InterPro
IPR005302 MoCF_Sase_C
IPR005303 MOSC_N
IPR011037 Pyrv_Knase-like_insert_dom_sf
PfamiView protein in Pfam
PF03473 MOSC, 1 hit
PF03476 MOSC_N, 1 hit
SUPFAMiSSF50800 SSF50800, 2 hits
PROSITEiView protein in PROSITE
PS51340 MOSC, 1 hit
ProtoNetiSearch...

Entry informationi

Entry nameiMARC2_HUMAN
AccessioniPrimary (citable) accession number: Q969Z3
Secondary accession number(s): B2D0R5
, D3DTB3, Q0JSK7, Q5VT67, Q5VXC7, Q7L317, Q9H066, Q9NWU0
Entry historyiIntegrated into UniProtKB/Swiss-Prot: January 23, 2007
Last sequence update: December 1, 2001
Last modified: September 12, 2018
This is version 135 of the entry and version 1 of the sequence. See 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
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