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Protein

Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial

Gene

Mccc1

Organism
Mus musculus (Mouse)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-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

Complete GO annotation...

Keywords - Molecular functioni

Ligase

Keywords - Ligandi

ATP-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 lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaSciurognathiMuroideaMuridaeMurinaeMusMus
Proteomesi
  • UP000000589 Componenti: Chromosome 3

Organism-specific databases

MGIiMGI:1919289. Mccc1.

Subcellular locationi

GO - Cellular componenti

Complete GO annotation...

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

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

Protein-protein interaction databases

BioGridi215108. 1 interactor.
IntActiQ99MR8. 5 interactors.
MINTiMINT-1844537.
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

Sequence similaritiesi

Contains 1 ATP-grasp domain.PROSITE-ProRule annotation
Contains 1 biotin carboxylation domain.Curated
Contains 1 biotinyl-binding domain.PROSITE-ProRule annotationCurated

Keywords - Domaini

Transit peptide

Phylogenomic databases

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

Family and domain databases

Gene3Di3.30.1490.20. 1 hit.
3.30.470.20. 1 hit.
3.40.50.20. 1 hit.
InterProiIPR011761. ATP-grasp.
IPR013815. ATP_grasp_subdomain_1.
IPR013816. ATP_grasp_subdomain_2.
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.
IPR011054. Rudment_hybrid_motif.
IPR011053. Single_hybrid_motif.
[Graphical view]
PfamiPF02785. Biotin_carb_C. 1 hit.
PF00289. Biotin_carb_N. 1 hit.
PF00364. Biotin_lipoyl. 1 hit.
PF02786. CPSase_L_D2. 1 hit.
[Graphical view]
SMARTiSM00878. Biotin_carb_C. 1 hit.
[Graphical view]
SUPFAMiSSF51230. SSF51230. 1 hit.
SSF51246. SSF51246. 1 hit.
SSF52440. SSF52440. 1 hit.
PROSITEiPS50975. ATP_GRASP. 1 hit.
PS50979. BC. 1 hit.
PS00188. BIOTIN. 1 hit.
PS50968. BIOTINYL_LIPOYL. 1 hit.
PS00867. CPSASE_2. 1 hit.
[Graphical view]

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.

Cross-referencesi

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.

3D structure databases

ProteinModelPortaliQ99MR8.
SMRiQ99MR8.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi215108. 1 interactor.
IntActiQ99MR8. 5 interactors.
MINTiMINT-1844537.
STRINGi10090.ENSMUSP00000029259.

PTM databases

iPTMnetiQ99MR8.
PhosphoSitePlusiQ99MR8.
SwissPalmiQ99MR8.

Proteomic databases

EPDiQ99MR8.
MaxQBiQ99MR8.
PaxDbiQ99MR8.
PeptideAtlasiQ99MR8.
PRIDEiQ99MR8.

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

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

Organism-specific databases

CTDi56922.
MGIiMGI:1919289. Mccc1.

Phylogenomic databases

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

Enzyme and pathway databases

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

Miscellaneous databases

ChiTaRSiMccc1. mouse.
PROiQ99MR8.
SOURCEiSearch...

Gene expression databases

BgeeiENSMUSG00000027709.
CleanExiMM_MCCC1.
ExpressionAtlasiQ99MR8. baseline and differential.
GenevisibleiQ99MR8. MM.

Family and domain databases

Gene3Di3.30.1490.20. 1 hit.
3.30.470.20. 1 hit.
3.40.50.20. 1 hit.
InterProiIPR011761. ATP-grasp.
IPR013815. ATP_grasp_subdomain_1.
IPR013816. ATP_grasp_subdomain_2.
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.
IPR011054. Rudment_hybrid_motif.
IPR011053. Single_hybrid_motif.
[Graphical view]
PfamiPF02785. Biotin_carb_C. 1 hit.
PF00289. Biotin_carb_N. 1 hit.
PF00364. Biotin_lipoyl. 1 hit.
PF02786. CPSase_L_D2. 1 hit.
[Graphical view]
SMARTiSM00878. Biotin_carb_C. 1 hit.
[Graphical view]
SUPFAMiSSF51230. SSF51230. 1 hit.
SSF51246. SSF51246. 1 hit.
SSF52440. SSF52440. 1 hit.
PROSITEiPS50975. ATP_GRASP. 1 hit.
PS50979. BC. 1 hit.
PS00188. BIOTIN. 1 hit.
PS50968. BIOTINYL_LIPOYL. 1 hit.
PS00867. CPSASE_2. 1 hit.
[Graphical view]
ProtoNetiSearch...

Entry informationi

Entry nameiMCCA_MOUSE
AccessioniPrimary (citable) accession number: Q99MR8
Secondary accession number(s): Q3TGU0, Q9D8R2
Entry historyi
Integrated into UniProtKB/Swiss-Prot: March 5, 2002
Last sequence update: March 5, 2002
Last modified: November 30, 2016
This is version 148 of the entry and version 2 of the sequence. [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

Similar proteinsi

Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into one UniRef entry.
90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.