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Protein

Methylcrotonoyl-CoA carboxylase beta chain, mitochondrial

Gene

Mccc2

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

Functioni

Carboxyltransferase subunit of the 3-methylcrotonyl-CoA carboxylase, an enzyme that catalyzes the conversion of 3-methylcrotonyl-CoA to 3-methylglutaconyl-CoA, a critical step for leucine and isovaleric acid catabolism.By similarity

Catalytic activityi

ATP + 3-methylcrotonoyl-CoA + HCO3- = ADP + phosphate + 3-methylglutaconyl-CoA.

Pathwayi: L-leucine degradation

This protein is involved in step 2 of the subpathway that synthesizes (S)-3-hydroxy-3-methylglutaryl-CoA from 3-isovaleryl-CoA.
Proteins known to be involved in the 3 steps of the subpathway in this organism are:
  1. Isovaleryl-CoA dehydrogenase, mitochondrial (Ivd)
  2. Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial (Mccc1), Methylcrotonoyl-CoA carboxylase beta chain, mitochondrial (Mccc2)
  3. Methylglutaconyl-CoA hydratase, mitochondrial (Auh)
This subpathway is part of the pathway L-leucine degradation, which is itself part of Amino-acid degradation.
View all proteins of this organism that are known to be involved in the subpathway that synthesizes (S)-3-hydroxy-3-methylglutaryl-CoA from 3-isovaleryl-CoA, the pathway L-leucine degradation and in Amino-acid degradation.

GO - Molecular functioni

GO - Biological processi

Keywordsi

Molecular functionLigase
LigandATP-binding, Nucleotide-binding

Enzyme and pathway databases

ReactomeiR-MMU-196780 Biotin transport and metabolism
R-MMU-70895 Branched-chain amino acid catabolism
UniPathwayiUPA00363; UER00861

Names & Taxonomyi

Protein namesi
Recommended name:
Methylcrotonoyl-CoA carboxylase beta chain, mitochondrial (EC:6.4.1.4)
Short name:
MCCase subunit beta
Alternative name(s):
3-methylcrotonyl-CoA carboxylase 2
3-methylcrotonyl-CoA carboxylase non-biotin-containing subunit
3-methylcrotonyl-CoA:carbon dioxide ligase subunit beta
Gene namesi
Name:Mccc2
OrganismiMus musculus (Mouse)
Taxonomic identifieri10090 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaMyomorphaMuroideaMuridaeMurinaeMusMus
Proteomesi
  • UP000000589 Componenti: Chromosome 13

Organism-specific databases

MGIiMGI:1925288 Mccc2

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 – 22MitochondrionBy similarityAdd BLAST22
ChainiPRO_000028406823 – 563Methylcrotonoyl-CoA carboxylase beta chain, mitochondrialAdd BLAST541

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei70N6-acetyllysine; alternateCombined sources1
Modified residuei70N6-succinyllysine; alternateCombined sources1
Modified residuei141N6-succinyllysineCombined sources1
Modified residuei433N6-succinyllysineCombined sources1
Modified residuei495N6-acetyllysine; alternateCombined sources1
Modified residuei495N6-succinyllysine; alternateCombined sources1
Modified residuei511N6-acetyllysineCombined sources1

Keywords - PTMi

Acetylation

Proteomic databases

EPDiQ3ULD5
MaxQBiQ3ULD5
PaxDbiQ3ULD5
PRIDEiQ3ULD5

PTM databases

iPTMnetiQ3ULD5
PhosphoSitePlusiQ3ULD5
SwissPalmiQ3ULD5

Expressioni

Gene expression databases

BgeeiENSMUSG00000021646
CleanExiMM_MCCC2
ExpressionAtlasiQ3ULD5 baseline and differential
GenevisibleiQ3ULD5 MM

Interactioni

Subunit structurei

Probably a dodecamer composed of six biotin-containing alpha subunits (MCCC1) and six beta (MCCC2) subunits.By similarity

Protein-protein interaction databases

BioGridi219115, 3 interactors
IntActiQ3ULD5, 3 interactors
MINTiQ3ULD5
STRINGi10090.ENSMUSP00000022148

Structurei

3D structure databases

ProteinModelPortaliQ3ULD5
SMRiQ3ULD5
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Domaini49 – 306CoA carboxyltransferase N-terminalPROSITE-ProRule annotationAdd BLAST258
Domaini309 – 555CoA carboxyltransferase C-terminalPROSITE-ProRule annotationAdd BLAST247

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Regioni49 – 555CarboxyltransferasePROSITE-ProRule annotationAdd BLAST507
Regioni343 – 372Acyl-CoA bindingSequence analysisAdd BLAST30

Sequence similaritiesi

Belongs to the AccD/PCCB family.Curated

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiKOG0540 Eukaryota
COG4799 LUCA
GeneTreeiENSGT00530000063337
HOGENOMiHOG000218692
HOVERGENiHBG052424
InParanoidiQ3ULD5
KOiK01969
OMAiMCGKAYD
OrthoDBiEOG091G05B5
PhylomeDBiQ3ULD5
TreeFamiTF300446

Family and domain databases

InterProiView protein in InterPro
IPR034733 AcCoA_carboxyl
IPR029045 ClpP/crotonase-like_dom_sf
IPR011763 COA_CT_C
IPR011762 COA_CT_N
PfamiView protein in Pfam
PF01039 Carboxyl_trans, 1 hit
SUPFAMiSSF52096 SSF52096, 2 hits
PROSITEiView protein in PROSITE
PS50989 COA_CT_CTER, 1 hit
PS50980 COA_CT_NTER, 1 hit

Sequencei

Sequence statusi: Complete.

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

Q3ULD5-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MWGALRSALR PCCRAAVPPQ RAYHGDSVAR LGTQPDSASS TYQENYEQMK
60 70 80 90 100
ALVSQLHERA QYVRLGGSEK ARARHTSRGK LLPRDRIDNL IDPGSPFLEF
110 120 130 140 150
SQFAGYQLYG DEEVPAGGII TGIGRVSGVE CMIVANDATV KGGTYYPVTV
160 170 180 190 200
KKHVRAQEIA LQNRLPCIYL VDSGGANLPR QADTFPDRDH FGRIFYNQAI
210 220 230 240 250
MSSKNITQIA VVMGSCTAGG AYVPAMADEN IIVQKQGTIF LAGPPLVKAA
260 270 280 290 300
TGEEVSAEDL GGADLHCRKS GVTDHYALDD HHALHLTRKV VRSLNYQKKM
310 320 330 340 350
DVTIEPSEEP LFPADELYGI VGANLKRSFD VREVIARIVD GSRFNEFKAL
360 370 380 390 400
YGDTLVTGFA RIFGYPVGII GNNGVLFSES AKKGAHFVQL CCQRNIPLLF
410 420 430 440 450
LQNITGFMVG RDYEAEGIAK DGAKMVAAVA CAKVPKITVI IGGSYGAGNY
460 470 480 490 500
GMCGRAYSPR FLYMWPNARI SVMGGEQAAT VLATVARDQK AREGKQFSSA
510 520 530 540 550
EEAALKEPII KRFEEEGNPY YSSARLWDDG IIDPVDTRLV LGLSLSAALN
560
APIQRTDFGI FRM
Length:563
Mass (Da):61,379
Last modified:October 11, 2005 - v1
Checksum:iB26486029F3B39AC
GO

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti115P → R in BAE25319 (PubMed:16141072).Curated1

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AK132265 mRNA Translation: BAE21068.1
AK143233 mRNA Translation: BAE25319.1
AK145564 mRNA Translation: BAE26513.1
AK146865 mRNA Translation: BAE27489.1
AK146844 mRNA Translation: BAE27475.1
CCDSiCCDS36764.1
RefSeqiNP_084302.1, NM_030026.2
UniGeneiMm.137327

Genome annotation databases

EnsembliENSMUST00000022148; ENSMUSP00000022148; ENSMUSG00000021646
GeneIDi78038
KEGGimmu:78038
UCSCiuc007rpz.1 mouse

Similar proteinsi

Entry informationi

Entry nameiMCCB_MOUSE
AccessioniPrimary (citable) accession number: Q3ULD5
Secondary accession number(s): Q3UPS6
Entry historyiIntegrated into UniProtKB/Swiss-Prot: April 17, 2007
Last sequence update: October 11, 2005
Last modified: May 23, 2018
This is version 102 of the entry and version 1 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. PATHWAY comments
    Index of metabolic and biosynthesis pathways
  3. SIMILARITY comments
    Index of protein domains and families

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