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UniProtKB - Q8MI68 (MUTA_PIG)
Protein
Methylmalonyl-CoA mutase, mitochondrial
Gene
MMUT
Organism
Sus scrofa (Pig)
Status
Functioni
Catalyzes the reversible isomerization of methylmalonyl-CoA (MMCoA) (generated from branched-chain amino acid metabolism and degradation of dietary odd chain fatty acids and cholesterol) to succinyl-CoA (3-carboxypropionyl-CoA), a key intermediate of the tricarboxylic acid cycle.
By similarityCatalytic activityi
Cofactori
adenosylcob(III)alaminBy similarity
Activity regulationi
Inhibited by itaconyl-CoA, a metabolite that inactivates the coenzyme B12 cofactor.By similarity
Sites
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Binding sitei | 50 | malonyl-CoABy similarity | 1 | |
Binding sitei | 228 | malonyl-CoABy similarity | 1 | |
Binding sitei | 255 | malonyl-CoABy similarity | 1 | |
Binding sitei | 265 | malonyl-CoABy similarity | 1 | |
Metal bindingi | 627 | Cobalt (adenosylcob(III)alamin axial ligand)By similarity | 1 |
GO - Molecular functioni
- cobalamin binding Source: UniProtKB
- GTPase activity Source: UniProtKB
- identical protein binding Source: UniProtKB
- metal ion binding Source: UniProtKB-KW
- methylmalonyl-CoA mutase activity Source: UniProtKB
- protein homodimerization activity Source: UniProtKB
Keywordsi
Molecular function | Isomerase |
Ligand | Cobalamin, Cobalt, Metal-binding |
Enzyme and pathway databases
BRENDAi | 5.4.99.2, 6170 |
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:MMUT Synonyms:MUT |
Organismi | Sus scrofa (Pig) |
Taxonomic identifieri | 9823 [NCBI] |
Taxonomic lineagei | Eukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Mammalia › Eutheria › Laurasiatheria › Artiodactyla › Suina › Suidae › Sus |
Proteomesi |
|
Subcellular locationi
Cytoplasm and Cytosol
- Cytoplasm By similarity
Mitochondrion
- Mitochondrion matrix By similarity
- Mitochondrion By similarity
Mitochondrion
- mitochondrial matrix Source: UniProtKB
- mitochondrion Source: UniProtKB
Other locations
- cytoplasm Source: UniProtKB
Keywords - Cellular componenti
Cytoplasm, MitochondrionPTM / Processingi
Molecule processing
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Transit peptidei | 1 – 32 | MitochondrionBy similarityAdd BLAST | 32 | |
ChainiPRO_0000019296 | 33 – 750 | Methylmalonyl-CoA mutase, mitochondrialAdd BLAST | 718 |
Amino acid modifications
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Modified residuei | 89 | N6-acetyllysineBy similarity | 1 | |
Modified residuei | 212 | N6-acetyllysineBy similarity | 1 | |
Modified residuei | 335 | N6-acetyllysineBy similarity | 1 | |
Modified residuei | 343 | N6-succinyllysineBy similarity | 1 | |
Modified residuei | 481 | PhosphoserineBy similarity | 1 | |
Modified residuei | 595 | N6-succinyllysineBy similarity | 1 | |
Modified residuei | 602 | N6-acetyllysineBy similarity | 1 |
Keywords - PTMi
Acetylation, PhosphoproteinProteomic databases
PaxDbi | Q8MI68 |
PeptideAtlasi | Q8MI68 |
PRIDEi | Q8MI68 |
Interactioni
Subunit structurei
Homodimer.
Interacts (the apoenzyme form) with MMAA; the interaction is GTP dependent.
By similarityGO - Molecular functioni
- identical protein binding Source: UniProtKB
- protein homodimerization activity Source: UniProtKB
Protein-protein interaction databases
STRINGi | 9823.ENSSSCP00000001889 |
Family & Domainsi
Domains and Repeats
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Domaini | 614 – 746 | B12-bindingPROSITE-ProRule annotationAdd BLAST | 133 |
Region
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Regioni | 96 – 99 | malonyl-CoA bindingBy similarity | 4 | |
Regioni | 106 – 110 | malonyl-CoA bindingBy similarity | 5 | |
Regioni | 216 – 218 | malonyl-CoA bindingBy similarity | 3 | |
Regioni | 304 – 306 | malonyl-CoA bindingBy similarity | 3 |
Sequence similaritiesi
Belongs to the methylmalonyl-CoA mutase family.Curated
Keywords - Domaini
Transit peptidePhylogenomic databases
eggNOGi | ENOG502QQ7X, Eukaryota |
InParanoidi | Q8MI68 |
OrthoDBi | 347581at2759 |
Family and domain databases
InterProi | View 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 |
Pfami | View protein in Pfam PF02310, B12-binding, 1 hit PF01642, MM_CoA_mutase, 1 hit |
SUPFAMi | SSF51703, SSF51703, 1 hit SSF52242, SSF52242, 1 hit |
TIGRFAMsi | TIGR00640, acid_CoA_mut_C, 1 hit TIGR00641, acid_CoA_mut_N, 1 hit |
PROSITEi | View protein in PROSITE PS51332, B12_BINDING, 1 hit PS00544, METMALONYL_COA_MUTASE, 1 hit |
i Sequence
Sequence statusi: Complete.
: The displayed sequence is further processed into a mature form. Sequence processingi
Q8MI68-1 [UniParc]FASTAAdd to basket
10 20 30 40 50
MLRAKTRLFL LSPHRLKQLK KSSGSRLVWQ RLLHQQQPLH PEWAALAKKQ
60 70 80 90 100
LKGKNPEELI WHTPEGISIK PLYSSRDTKD FPEELPGVKP FTRGPYPTMY
110 120 130 140 150
TFRPWTIRQY AGFSTVEESN KFYKDIIKAG QQGLSVAFDL ATHRGYDSDN
160 170 180 190 200
PRVHGDVGMA GVAIDTVEDT KILFDGIPLE KMSVSMTMNG AVIPVLATFI
210 220 230 240 250
VSGEEQGVPK EKLTGTIQND ILKEFMVRNT YIFPPEPSMK IIADIFQYTA
260 270 280 290 300
KHMPKFNSIS ISGYHMQEAG ADAILELAYT IADGLEYCRT GLQAGLTIDE
310 320 330 340 350
FAPRLSFFWG IGMNFYMEIA KMRAGRRLWA HLIEKMFRRK NSKSLLLRAH
360 370 380 390 400
CQTSGWSLTE QDPYNNIIRT TVEAMAAVFG GTQSLHTNSF DEALGLPTVK
410 420 430 440 450
SARIARNTQI IIQEESGIPK VADPWGGSYM MESLTNDVYD AALKLINEIE
460 470 480 490 500
EMGGMAKAVA EGIPKLRIEE CAARRQARID SGSEVIVGVN KYQLEKEESV
510 520 530 540 550
EVLAIDNTSV RNKQIEKLKK VNPAGIKLWL ERCLTALTAC AASGDGNILA
560 570 580 590 600
LAVEATRARC TVGEITDAMK KVFGEHKAND RMVSGAYRQE FGESKEISFA
610 620 630 640 650
IKRVHKFMER EGRRPRLLVA KMGQDGHDRG AKVIATGFAD LGFDVDIGPL
660 670 680 690 700
FQTPREVAQQ AVDADVHAVG VSTLAAGHKT LVPELIKELS TLGRPDILVM
710 720 730 740 750
CGGVIPPQDY EFLFEVGVSN VFGPGTRIPK AAVQVLNDIE KCLEKKQQSM
Experimental Info
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Sequence conflicti | 339 | R → P in CAD43288 (Ref. 1) Curated | 1 |
Sequence databases
Select the link destinations: EMBLi GenBanki DDBJi Links Updated | AJ496801 mRNA Translation: CAD43173.1 AJ504726 Genomic DNA Translation: CAD43288.1 |
RefSeqi | NP_999570.1, NM_214405.1 |
Genome annotation databases
GeneIDi | 399535 |
KEGGi | ssc:399535 |
Similar proteinsi
Cross-referencesi
Sequence databases
Select the link destinations: EMBLi GenBanki DDBJi Links Updated | AJ496801 mRNA Translation: CAD43173.1 AJ504726 Genomic DNA Translation: CAD43288.1 |
RefSeqi | NP_999570.1, NM_214405.1 |
3D structure databases
AlphaFoldDBi | Q8MI68 |
SMRi | Q8MI68 |
ModBasei | Search... |
Protein-protein interaction databases
STRINGi | 9823.ENSSSCP00000001889 |
Proteomic databases
PaxDbi | Q8MI68 |
PeptideAtlasi | Q8MI68 |
PRIDEi | Q8MI68 |
Genome annotation databases
GeneIDi | 399535 |
KEGGi | ssc:399535 |
Organism-specific databases
CTDi | 4594 |
Phylogenomic databases
eggNOGi | ENOG502QQ7X, Eukaryota |
InParanoidi | Q8MI68 |
OrthoDBi | 347581at2759 |
Enzyme and pathway databases
BRENDAi | 5.4.99.2, 6170 |
Family and domain databases
InterProi | View 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 |
Pfami | View protein in Pfam PF02310, B12-binding, 1 hit PF01642, MM_CoA_mutase, 1 hit |
SUPFAMi | SSF51703, SSF51703, 1 hit SSF52242, SSF52242, 1 hit |
TIGRFAMsi | TIGR00640, acid_CoA_mut_C, 1 hit TIGR00641, acid_CoA_mut_N, 1 hit |
PROSITEi | View protein in PROSITE PS51332, B12_BINDING, 1 hit PS00544, METMALONYL_COA_MUTASE, 1 hit |
MobiDBi | Search... |
Entry informationi
Entry namei | MUTA_PIG | |
Accessioni | Q8MI68Primary (citable) accession number: Q8MI68 Secondary accession number(s): Q8MI71 | |
Entry historyi | Integrated into UniProtKB/Swiss-Prot: | January 16, 2004 |
Last sequence update: | May 10, 2005 | |
Last modified: | May 25, 2022 | |
This is version 100 of the entry and version 2 of the sequence. See complete history. | ||
Entry statusi | Reviewed (UniProtKB/Swiss-Prot) | |
Annotation program | Chordata Protein Annotation Program |
Miscellaneousi
Keywords - Technical termi
Reference proteomeDocuments
- SIMILARITY comments
Index of protein domains and families