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Protein

Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial

Gene

Mccc1

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

Functioni

Biotin-attachment 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.

Cofactori

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.

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Binding sitei159ATPBy similarity1
Binding sitei243ATPBy similarity1
Binding sitei278ATPBy similarity1
Active sitei335By similarity1

GO - Molecular functioni

GO - Biological processi

Keywordsi

Molecular functionLigase
LigandATP-binding, Biotin, 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 subunit alpha, mitochondrial (EC:6.4.1.4)
Short name:
MCCase subunit alpha
Alternative name(s):
3-methylcrotonyl-CoA carboxylase 1
3-methylcrotonyl-CoA carboxylase biotin-containing subunit
3-methylcrotonyl-CoA:carbon dioxide ligase subunit alpha
Gene namesi
Name:Mccc1
Synonyms:Mcca
OrganismiMus musculus (Mouse)
Taxonomic identifieri10090 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaMyomorphaMuroideaMuridaeMurinaeMusMus
Proteomesi
  • UP000000589 Componenti: Chromosome 3

Organism-specific databases

MGIiMGI:1919289 Mccc1

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 – 38MitochondrionBy similarityAdd BLAST38
ChainiPRO_000000283439 – 717Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrialAdd BLAST679

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Modified residuei180N6-acetyllysineCombined sources1
Modified residuei193N6-acetyllysineCombined sources1
Modified residuei233N6-acetyllysineCombined sources1
Modified residuei490N6-acetyllysineCombined sources1
Modified residuei577N6-acetyllysine; alternateCombined sources1
Modified residuei577N6-succinyllysine; alternateCombined sources1
Modified residuei677N6-biotinyllysinePROSITE-ProRule annotationBy similarity1

Post-translational modificationi

Acetylated.1 Publication

Keywords - PTMi

Acetylation

Proteomic databases

EPDiQ99MR8
MaxQBiQ99MR8
PaxDbiQ99MR8
PeptideAtlasiQ99MR8
PRIDEiQ99MR8

PTM databases

iPTMnetiQ99MR8
PhosphoSitePlusiQ99MR8
SwissPalmiQ99MR8

Expressioni

Gene expression databases

BgeeiENSMUSG00000027709
CleanExiMM_MCCC1
ExpressionAtlasiQ99MR8 baseline and differential
GenevisibleiQ99MR8 MM

Interactioni

Subunit structurei

Probably a dodecamer composed of six biotin-containing alpha subunits (MCCC1) and six beta (MCCC2) subunits (By similarity). Interacts (via the biotin carboxylation domain) with SIRT4 (PubMed:23438705).By similarity1 Publication

Protein-protein interaction databases

BioGridi215108, 3 interactors
IntActiQ99MR8, 8 interactors
MINTiQ99MR8
STRINGi10090.ENSMUSP00000029259

Structurei

3D structure databases

ProteinModelPortaliQ99MR8
SMRiQ99MR8
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Domaini45 – 490Biotin carboxylationAdd BLAST446
Domaini163 – 360ATP-graspPROSITE-ProRule annotationAdd BLAST198
Domaini622 – 711Biotinyl-bindingPROSITE-ProRule annotationAdd BLAST90

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiKOG0238 Eukaryota
COG4770 LUCA
GeneTreeiENSGT00550000074675
HOGENOMiHOG000008989
HOVERGENiHBG000555
InParanoidiQ99MR8
KOiK01968
OMAiFVEICSH
OrthoDBiEOG091G06RG
PhylomeDBiQ99MR8
TreeFamiTF105650

Family and domain databases

Gene3Di3.30.1490.20, 1 hit
InterProiView protein in InterPro
IPR011761 ATP-grasp
IPR013815 ATP_grasp_subdomain_1
IPR005481 BC-like_N
IPR001882 Biotin_BS
IPR011764 Biotin_carboxylation_dom
IPR005482 Biotin_COase_C
IPR000089 Biotin_lipoyl
IPR005479 CbamoylP_synth_lsu-like_ATP-bd
IPR016185 PreATP-grasp_dom_sf
IPR011054 Rudment_hybrid_motif
IPR011053 Single_hybrid_motif
PfamiView protein in Pfam
PF02785 Biotin_carb_C, 1 hit
PF00289 Biotin_carb_N, 1 hit
PF00364 Biotin_lipoyl, 1 hit
PF02786 CPSase_L_D2, 1 hit
SMARTiView protein in SMART
SM00878 Biotin_carb_C, 1 hit
SUPFAMiSSF51230 SSF51230, 1 hit
SSF51246 SSF51246, 1 hit
SSF52440 SSF52440, 1 hit
PROSITEiView protein in PROSITE
PS50975 ATP_GRASP, 1 hit
PS50979 BC, 1 hit
PS00188 BIOTIN, 1 hit
PS50968 BIOTINYL_LIPOYL, 1 hit
PS00867 CPSASE_2, 1 hit

Sequencei

Sequence statusi: Complete.

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

Q99MR8-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MAAAALLAAV DRNQLRRVPI LLLQPREWAW KLRTMKYGTT PGGSITKVLI
60 70 80 90 100
ANRGEIACRV IRTAKKMGVQ SVAVYSEADR NSMHVDMADE AYSIGPAPSQ
110 120 130 140 150
QSYLAMEKII QVAKSSAAQA IHPGYGFLSE NMEFAELCKQ EGIIFIGPPS
160 170 180 190 200
SAIRDMGIKS TSKSIMAAAG VPVVEGYHGK DQSDQCLREH AGKIGYPVMI
210 220 230 240 250
KAVRGGGGKG MRIVRSEREF QEQLESARRE AKKSFNDDAM LIEKFVDTPR
260 270 280 290 300
HVEVQVFGDH HGNAVYLFER DCSVQRRHQK IIEEAPAPGI NPEVRRKLGE
310 320 330 340 350
AAVRAAKAVK YVGAGTVEFI MDSRHNFYFM EMNTRLQVEH PVTEMITGTD
360 370 380 390 400
LVEWQLRIAA GEKIPLSQEE IPLQGHAFEA RIYAEDPDNN FMPGAGPLVH
410 420 430 440 450
LSTPSADMST RIETGVRQGD EVSVHYDPMI AKLVVWASDR QSALSKLRYC
460 470 480 490 500
LHQYNIVGLR SNVDFLLRLS GHPEFEAGNV HTDFIPQHHK DLLPSHSTIA
510 520 530 540 550
KESVCQAALG LILKEKEMTS AFKLHTQDQF SPFSFSSGRR LNISYTRNMT
560 570 580 590 600
LRSGKSDIVI AVTYNRDGSY DMQIDNKSFR VLGDLSSEDG CTYLKSSING
610 620 630 640 650
VARKSKFILL DNTVHLFSME GSIEVGIPVP KYLSPVSAEG AQGGTIAPMT
660 670 680 690 700
GTIEKVFVKA GDRVKAGDSL MVMIAMKMEH TIKAPKDGRI KKVFFSEGAQ
710
ANRHAPLVEF EEEESDK
Length:717
Mass (Da):79,344
Last modified:March 5, 2002 - v2
Checksum:iF653FE7AC1E5AA90
GO

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Sequence conflicti324R → K in AAH21382 (PubMed:15489334).Curated1
Sequence conflicti507A → P in AAG50244 (PubMed:11181649).Curated1

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AF310338 mRNA Translation: AAG50244.1
AK007782 mRNA Translation: BAB25253.1
AK031072 mRNA Translation: BAC27239.1
AK168589 mRNA Translation: BAE40458.1
BC021382 mRNA Translation: AAH21382.1
CCDSiCCDS17307.1
RefSeqiNP_076133.3, NM_023644.4
UniGeneiMm.249016

Genome annotation databases

EnsembliENSMUST00000029259; ENSMUSP00000029259; ENSMUSG00000027709
GeneIDi72039
KEGGimmu:72039
UCSCiuc008oyy.3 mouse

Similar proteinsi

Entry informationi

Entry nameiMCCA_MOUSE
AccessioniPrimary (citable) accession number: Q99MR8
Secondary accession number(s): Q3TGU0, Q9D8R2
Entry historyiIntegrated into UniProtKB/Swiss-Prot: March 5, 2002
Last sequence update: March 5, 2002
Last modified: May 23, 2018
This is version 161 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

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