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Protein

Methylmalonyl-CoA mutase, mitochondrial

Gene

Mut

Organism
Mus musculus (Mouse)
Status
Reviewed-Annotation score: -Experimental evidence at protein leveli

Functioni

Involved in the degradation of several amino acids, odd-chain fatty acids and cholesterol via propionyl-CoA to the tricarboxylic acid cycle.By similarity

Catalytic activityi

(R)-methylmalonyl-CoA = succinyl-CoA.By similarity

Cofactori

adenosylcob(III)alaminBy similarity

Enzyme regulationi

Inhibited by itaconyl-CoA, a metabolite that inactivates the coenzyme B12 cofactor.By similarity

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Binding sitei48malonyl-CoABy similarity1
Binding sitei226malonyl-CoABy similarity1
Binding sitei253malonyl-CoABy similarity1
Binding sitei263malonyl-CoABy similarity1
Metal bindingi625Cobalt (cobalamin 2 axial ligand)By similarity1

GO - Molecular functioni

GO - Biological processi

  • homocysteine metabolic process Source: MGI
  • positive regulation of GTPase activity Source: MGI
  • post-embryonic development Source: MGI

Keywordsi

Molecular functionIsomerase
LigandCobalamin, Cobalt, Metal-binding

Enzyme and pathway databases

ReactomeiR-MMU-196741 Cobalamin (Cbl, vitamin B12) transport and metabolism
R-MMU-71032 Propionyl-CoA catabolism

Names & Taxonomyi

Protein namesi
Recommended name:
Methylmalonyl-CoA mutase, mitochondrial (EC:5.4.99.2By similarity)
Short name:
MCM
Alternative name(s):
Methylmalonyl-CoA isomerase
Gene namesi
Name:Mut
OrganismiMus musculus (Mouse)
Taxonomic identifieri10090 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaMyomorphaMuroideaMuridaeMurinaeMusMus
Proteomesi
  • UP000000589 Componenti: Chromosome 17

Organism-specific databases

MGIiMGI:97239 Mut

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

Mitochondrion

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transit peptidei1 – 30MitochondrionBy similarityAdd BLAST30
ChainiPRO_000001929531 – 748Methylmalonyl-CoA mutase, mitochondrialAdd BLAST718

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei87N6-acetyllysineCombined sources1
Modified residuei210N6-acetyllysineCombined sources1
Modified residuei333N6-acetyllysineCombined sources1
Modified residuei341N6-succinyllysineCombined sources1
Modified residuei479PhosphoserineBy similarity1
Modified residuei593N6-succinyllysineCombined sources1
Modified residuei600N6-acetyllysineCombined sources1

Keywords - PTMi

Acetylation, Phosphoprotein

Proteomic databases

EPDiP16332
MaxQBiP16332
PaxDbiP16332
PeptideAtlasiP16332
PRIDEiP16332

PTM databases

iPTMnetiP16332
PhosphoSitePlusiP16332
SwissPalmiP16332

Expressioni

Gene expression databases

BgeeiENSMUSG00000023921
CleanExiMM_MUT
GenevisibleiP16332 MM

Interactioni

Subunit structurei

Homodimer. Interacts (the apoenzyme form) with MMAA; the interaction is GTP dependent.By similarity

GO - Molecular functioni

Protein-protein interaction databases

IntActiP16332, 3 interactors
MINTiP16332
STRINGi10090.ENSMUSP00000130941

Structurei

3D structure databases

ProteinModelPortaliP16332
SMRiP16332
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Domaini612 – 744B12-bindingPROSITE-ProRule annotationAdd BLAST133

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Regioni94 – 97malonyl-CoA bindingBy similarity4
Regioni104 – 108malonyl-CoA bindingBy similarity5
Regioni214 – 216malonyl-CoA bindingBy similarity3
Regioni302 – 304malonyl-CoA bindingBy similarity3

Sequence similaritiesi

Belongs to the methylmalonyl-CoA mutase family.Curated

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiENOG410IE7I Eukaryota
COG1884 LUCA
COG2185 LUCA
GeneTreeiENSGT00390000011892
HOGENOMiHOG000003917
HOVERGENiHBG006423
InParanoidiP16332
KOiK01847
OMAiPLFQTPR
OrthoDBiEOG091G02GM
TreeFamiTF313557

Family and domain databases

InterProiView protein in InterPro
IPR006159 Acid_CoA_mut_C
IPR016176 Cbl-dep_enz_cat
IPR006158 Cobalamin-bd
IPR036724 Cobalamin-bd_sf
IPR006099 MeMalonylCoA_mutase_a/b_cat
IPR006098 MMCoA_mutase_a_cat
PfamiView protein in Pfam
PF02310 B12-binding, 1 hit
PF01642 MM_CoA_mutase, 1 hit
SUPFAMiSSF51703 SSF51703, 1 hit
SSF52242 SSF52242, 1 hit
TIGRFAMsiTIGR00640 acid_CoA_mut_C, 1 hit
TIGR00641 acid_CoA_mut_N, 1 hit
PROSITEiView protein in PROSITE
PS51332 B12_BINDING, 1 hit
PS00544 METMALONYL_COA_MUTASE, 1 hit

Sequencei

Sequence statusi: Complete.

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

P16332-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MLRAKNQLFL LSPHYLKQLN IPSASRWKRL LHQQQPLHPE WAVLAKKQLK
60 70 80 90 100
GKNPEDLIWH TPEGISIKPL YSRADTLDLP EELPGVKPFT RGPYPTMYTY
110 120 130 140 150
RPWTIRQYAG FSTVEESNKF YKDNIKAGQQ GLSVAFDLAT HRGYDSDNPR
160 170 180 190 200
VRGDVGMAGV AIDTVEDTKI LFDGIPLEKM SVSMTMNGAV IPVLATFIVT
210 220 230 240 250
GEEQGVPKEK LTGTIQNDIL KEFMVRNTYI FPPEPSMKII ADIFQYTAQH
260 270 280 290 300
MPKFNSISIS GYHMQEAGAD AILELAYTIA DGLEYCRTGL QAGLTIDEFA
310 320 330 340 350
PRLSFFWGIG MNFYMEIAKM RAGRRLWAHL IEKMFQPKNS KSLLLRAHCQ
360 370 380 390 400
TSGWSLTEQD PYNNIVRTAI EAMAAVFGGT QSLHTNSFDE ALGLPTVKSA
410 420 430 440 450
RIARNTQIII QEESGIPKVA DPWGGSYMME SLTNDVYEAA LKLIYEVEEM
460 470 480 490 500
GGMAKAVAEG IPKLRIEECA ARRQARIDSG SEVIVGVNKY QLEKEDSVEV
510 520 530 540 550
LAIDNTSVRK KQIEKLKKIK SSRDQALAEQ CLSALTQCAA SGDGNILALA
560 570 580 590 600
VDAARARCTV GEITDALKKV FGEHKANDRM VSGAYRQEFG ESKEITSAIK
610 620 630 640 650
RVNKFMEREG RRPRLLVAKM GQDGHDRGAK VIATGFADLG FDVDIGPLFQ
660 670 680 690 700
TPREVAQQAV DADVHAVGVS TLAAGHKTLV PELIKELTAL GRPDILVMCG
710 720 730 740
GVIPPQDYEF LYEVGVSNVF GPGTRIPRAA VQVLDDIEKC LAEKQQSV
Length:748
Mass (Da):82,844
Last modified:July 27, 2011 - v2
Checksum:iDF4A62100A8BD743
GO

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti256S → P in CAA36204 (PubMed:1978672).Curated1
Sequence conflicti269A → T in CAA36204 (PubMed:1978672).Curated1
Sequence conflicti385T → S in CAA36204 (PubMed:1978672).Curated1
Sequence conflicti407Q → R in CAA36204 (PubMed:1978672).Curated1
Sequence conflicti491Q → H in CAA36204 (PubMed:1978672).Curated1
Sequence conflicti499 – 508EVLAIDNTSV → HLLAIDIISL in CAA36204 (PubMed:1978672).Curated10
Sequence conflicti561G → P in CAA36204 (PubMed:1978672).Curated1
Sequence conflicti567L → F in CAA36204 (PubMed:1978672).Curated1
Sequence conflicti613 – 614PR → LG in CAA36204 (PubMed:1978672).Curated2
Sequence conflicti622Q → K in CAA36204 (PubMed:1978672).Curated1
Sequence conflicti657 – 658QQ → HD in CAA36204 (PubMed:1978672).Curated2
Sequence conflicti672L → H in CAA36204 (PubMed:1978672).Curated1

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
X51941 mRNA Translation: CAA36204.1
AK146309 mRNA Translation: BAE27064.1
CH466559 Genomic DNA Translation: EDL23389.1
CCDSiCCDS37618.1
PIRiS08680
RefSeqiNP_032676.2, NM_008650.3
UniGeneiMm.259884

Genome annotation databases

EnsembliENSMUST00000169611; ENSMUSP00000130941; ENSMUSG00000023921
GeneIDi17850
KEGGimmu:17850
UCSCiuc008coo.2 mouse

Similar proteinsi

Entry informationi

Entry nameiMUTA_MOUSE
AccessioniPrimary (citable) accession number: P16332
Secondary accession number(s): Q3UJU1
Entry historyiIntegrated into UniProtKB/Swiss-Prot: August 1, 1990
Last sequence update: July 27, 2011
Last modified: May 23, 2018
This is version 146 of the entry and version 2 of the sequence. See complete history.
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Miscellaneousi

Keywords - Technical termi

Complete proteome, Reference proteome

Documents

  1. MGD cross-references
    Mouse Genome Database (MGD) cross-references in UniProtKB/Swiss-Prot
  2. SIMILARITY comments
    Index of protein domains and families
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