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Protein

Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial

Gene

Mccc1

Organism
Rattus norvegicus (Rat)
Status
Reviewed-Annotation score: Annotation score: 4 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.By similarity

Cofactori

biotinBy similarity

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 beta chain, mitochondrial (Mccc2), Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial (Mccc1)
  3. no protein annotated in this organism
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)LengthDescriptionGraphical viewFeature identifierActions
Binding sitei159 – 1591ATPBy similarity
Binding sitei243 – 2431ATPBy similarity
Binding sitei278 – 2781ATPBy similarity
Active sitei335 – 3351By similarity

GO - Molecular functioni

GO - Biological processi

Complete GO annotation...

Keywords - Molecular functioni

Ligase

Keywords - Ligandi

ATP-binding, Biotin, Nucleotide-binding

Enzyme and pathway databases

BRENDAi6.4.1.4. 5301.
UniPathwayiUPA00363; UER00861.

Names & Taxonomyi

Protein namesi
Recommended name:
Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrialBy similarity (EC:6.4.1.4)
Short name:
MCCase subunit alphaBy similarity
Alternative name(s):
3-methylcrotonyl-CoA carboxylase 1By similarity
3-methylcrotonyl-CoA carboxylase biotin-containing subunitBy similarity
3-methylcrotonyl-CoA:carbon dioxide ligase subunit alphaBy similarity
Gene namesi
Name:Mccc1Imported
Synonyms:MccaBy similarity
OrganismiRattus norvegicus (Rat)
Taxonomic identifieri10116 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaSciurognathiMuroideaMuridaeMurinaeRattus
Proteomesi
  • UP000002494 Componenti: Unplaced

Organism-specific databases

RGDi1310615. Mccc1.

Subcellular locationi

  • Mitochondrion matrix By similarity

GO - Cellular componenti

Complete GO annotation...

Keywords - Cellular componenti

Mitochondrion

PTM / Processingi

Molecule processing

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Transit peptidei1 – 3838MitochondrionBy similarityAdd
BLAST
Chaini39 – 715677Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrialPRO_0000365095Add
BLAST

Amino acid modifications

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Modified residuei193 – 1931N6-acetyllysineBy similarity
Modified residuei233 – 2331N6-acetyllysineBy similarity
Modified residuei490 – 4901N6-acetyllysineBy similarity
Modified residuei577 – 5771N6-acetyllysine; alternateBy similarity
Modified residuei577 – 5771N6-succinyllysine; alternateBy similarity
Modified residuei677 – 6771N6-biotinyllysinePROSITE-ProRule annotationBy similarity

Keywords - PTMi

Acetylation

Proteomic databases

PaxDbiQ5I0C3.
PRIDEiQ5I0C3.

PTM databases

iPTMnetiQ5I0C3.
PhosphoSiteiQ5I0C3.

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

STRINGi10116.ENSRNOP00000018942.

Structurei

3D structure databases

ProteinModelPortaliQ5I0C3.
SMRiQ5I0C3. Positions 641-714.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Domaini45 – 490446Biotin carboxylationSequence analysisAdd
BLAST
Domaini163 – 360198ATP-graspPROSITE-ProRule annotationAdd
BLAST
Domaini622 – 71190Biotinyl-bindingPROSITE-ProRule annotationAdd
BLAST

Sequence similaritiesi

Contains 1 ATP-grasp domain.PROSITE-ProRule annotation
Contains 1 biotin carboxylation domain.Sequence analysis
Contains 1 biotinyl-binding domain.PROSITE-ProRule annotation

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiKOG0238. Eukaryota.
COG4770. LUCA.
HOGENOMiHOG000008989.
HOVERGENiHBG000555.
InParanoidiQ5I0C3.
KOiK01968.
PhylomeDBiQ5I0C3.

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.

Q5I0C3-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MAAAALLAAV DRNQLRRVPI LLLQPREWPW KHRTVKYGTT PGGSITKVLI
60 70 80 90 100
ANRGEIACRV IRTARKMGVQ SVAVYSEADR NSMHVDMADE AYSIGPAPSQ
110 120 130 140 150
QSYLAMEKII QVAKSSAAQA IHPGYGFLSE NMEFAEFCKQ EGIIFIGPPS
160 170 180 190 200
TAIRDMGIKS TSKSIMAAAG VPVVEGYHGN DQSDECLKEH AGKIGYPVMI
210 220 230 240 250
KAIRGGGGKG MRIIRSEKEF QEQLESARRE AKKSFNDDAM LIEKFVDTPR
260 270 280 290 300
HVEVQVFGDH HGNAVYLFER DCSVQRRHQK IIEEAPAPGI DPEVRRRLGE
310 320 330 340 350
AAVRAAKAVN YVGAGTVEFI MDSKHNFYFM EMNTRLQVEH PVTEMITGTD
360 370 380 390 400
LVEWQLRIAA GEKIPLSQEE IPLQGHAFEA RIYAEDPDNN FMPGAGPLVH
410 420 430 440 450
LSTPPPDMST RIETGVRQGD EVSVHYDPMI AKLVVWASDR QSALSKLRYS
460 470 480 490 500
LHQYNIVGLR TNVDFLLRLS GHSEFEAGNV HTDFIPQHHK DLLPTHSTIA
510 520 530 540 550
KESVCQAALG LILKEKEMTS AFKLHTQDQF SPFSFSSGRR LNISYTRNMT
560 570 580 590 600
LRSGKNDIII AVTYNRDGSY DMQIENKLFR VLGDLSNEDG YTYLKSSVNG
610 620 630 640 650
VASKSKFILL DNTIYLFSME GSIEVGIPVP KYLSPVSAEG TQGGTIAPMT
660 670 680 690 700
GTIEKVFVKA GDRVKAGDAL MVMIAMKMEH TIKAPKDGRI KKVFFSEGAQ
710
ANRHAPLVEF EEEEV
Length:715
Mass (Da):79,330
Last modified:February 15, 2005 - v1
Checksum:i20FB2EAB8664AD8F
GO

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
BC088473 mRNA. Translation: AAH88473.1.
RefSeqiNP_001009653.1. NM_001009653.1.
UniGeneiRn.15587.

Genome annotation databases

GeneIDi294972.
KEGGirno:294972.
UCSCiRGD:1310615. rat.

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
BC088473 mRNA. Translation: AAH88473.1.
RefSeqiNP_001009653.1. NM_001009653.1.
UniGeneiRn.15587.

3D structure databases

ProteinModelPortaliQ5I0C3.
SMRiQ5I0C3. Positions 641-714.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

STRINGi10116.ENSRNOP00000018942.

PTM databases

iPTMnetiQ5I0C3.
PhosphoSiteiQ5I0C3.

Proteomic databases

PaxDbiQ5I0C3.
PRIDEiQ5I0C3.

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

GeneIDi294972.
KEGGirno:294972.
UCSCiRGD:1310615. rat.

Organism-specific databases

CTDi56922.
RGDi1310615. Mccc1.

Phylogenomic databases

eggNOGiKOG0238. Eukaryota.
COG4770. LUCA.
HOGENOMiHOG000008989.
HOVERGENiHBG000555.
InParanoidiQ5I0C3.
KOiK01968.
PhylomeDBiQ5I0C3.

Enzyme and pathway databases

UniPathwayiUPA00363; UER00861.
BRENDAi6.4.1.4. 5301.

Miscellaneous databases

PROiQ5I0C3.

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_RAT
AccessioniPrimary (citable) accession number: Q5I0C3
Entry historyi
Integrated into UniProtKB/Swiss-Prot: March 3, 2009
Last sequence update: February 15, 2005
Last modified: September 7, 2016
This is version 93 of the entry and version 1 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. PATHWAY comments
    Index of metabolic and biosynthesis pathways
  2. 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.