Reviewed, UniProtKB/Swiss-Prot Q5I0C3 (MCCA_RAT)

Names and origin

Protein names

Recommended name:
    Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial
      Short name=MCCase subunit alpha
    EC=6.4.1.4
Alternative name(s):
    3-methylcrotonyl-CoA carboxylase 1
    3-methylcrotonyl-CoA:carbon dioxide ligase subunit alpha
    3-methylcrotonyl-CoA carboxylase biotin-containing subunit

Gene names

Name:	Mccc1
Synonyms:	Mcca

Organism

Rattus norvegicus (Rat)

Taxonomic identifier

10116 [NCBI]

Taxonomic lineage

Eukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Mammalia › Eutheria › Euarchontoglires › Glires › Rodentia › Sciurognathi › Muroidea › Muridae › Murinae › Rattus

Protein attributes

Sequence length	715 AA.
Sequence status	Complete.
Sequence processing	The displayed sequence is further processed into a mature form.
Protein existence	Evidence at protein level.

General annotation (Comments)

Catalytic activity	ATP + 3-methylcrotonoyl-CoA + HCO3- = ADP + phosphate + 3-methylglutaconyl-CoA. UniProtKB Q96RQ3
Cofactor	Biotin By similarity. UniProtKB Q96RQ3
Pathway	Amino-acid degradation; L-leucine degradation; (S)-3-hydroxy-3-methylglutaryl-CoA from 3-isovaleryl-CoA: step 2/3.
Subunit structure	Probably a dodecamer composed of six biotin-containing alpha subunits and six beta subunits By similarity. UniProtKB Q96RQ3
Subcellular location	Mitochondrion matrix By similarity UniProtKB Q96RQ3.
Sequence similarities	Contains 1 ATP-grasp domain. Contains 1 biotin carboxylation domain. Contains 1 biotinyl-binding domain.

Ontologies

Keywords
Cellular component	Mitochondrion
Domain	Transit peptide
Ligand	ATP-binding Biotin Nucleotide-binding
Molecular function	Ligase
Gene Ontology (GO)
Biological process	metabolic process Inferred from electronic annotation. Source: InterPro
Cellular component	mitochondrial matrix Inferred from electronic annotation. Source: UniProtKB-SubCell
Molecular function	ATP binding Inferred from electronic annotation. Source: UniProtKB-KW biotin binding Inferred from electronic annotation. Source: InterPro methylcrotonoyl-CoA carboxylase activity Inferred from electronic annotation. Source: EC
Complete GO annotation...

Sequence annotation (Features)

	Feature key	Position(s)	Length	Description	Graphical view	Feature identifier
Molecule processing
	Transit peptide	1 – 44	44	Mitochondrion By similarity UniProtKB Q99MR8
	Chain	45 – 715	671	Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial UniProtKB Q99MR8		PRO_0000365095
Regions
	Domain	45 – 490	446	Biotin carboxylation
	Domain	163 – 360	198	ATP-grasp
	Domain	625 – 710	86	Biotinyl-binding
Sites
	Active site	335	1	By similarity UniProtKB P24182
	Binding site	159	1	ATP By similarity UniProtKB P24182
	Binding site	243	1	ATP By similarity UniProtKB P24182
	Binding site	278	1	ATP By similarity UniProtKB P24182
Amino acid modifications
	Modified residue	677	1	N6-biotinyllysine By similarity

Sequences

	Sequence		Length	Mass (Da)
	Q5I0C3-1 [UniParc]. Last modified February 15, 2005. Version 1. Checksum: 20FB2EAB8664AD8F	FASTA	715	79,330
	10 20 30 40 50 60 MAAAALLAAV DRNQLRRVPI LLLQPREWPW KHRTVKYGTT PGGSITKVLI ANRGEIACRV 70 80 90 100 110 120 IRTARKMGVQ SVAVYSEADR NSMHVDMADE AYSIGPAPSQ QSYLAMEKII QVAKSSAAQA 130 140 150 160 170 180 IHPGYGFLSE NMEFAEFCKQ EGIIFIGPPS TAIRDMGIKS TSKSIMAAAG VPVVEGYHGN 190 200 210 220 230 240 DQSDECLKEH AGKIGYPVMI KAIRGGGGKG MRIIRSEKEF QEQLESARRE AKKSFNDDAM 250 260 270 280 290 300 LIEKFVDTPR HVEVQVFGDH HGNAVYLFER DCSVQRRHQK IIEEAPAPGI DPEVRRRLGE 310 320 330 340 350 360 AAVRAAKAVN YVGAGTVEFI MDSKHNFYFM EMNTRLQVEH PVTEMITGTD LVEWQLRIAA 370 380 390 400 410 420 GEKIPLSQEE IPLQGHAFEA RIYAEDPDNN FMPGAGPLVH LSTPPPDMST RIETGVRQGD 430 440 450 460 470 480 EVSVHYDPMI AKLVVWASDR QSALSKLRYS LHQYNIVGLR TNVDFLLRLS GHSEFEAGNV 490 500 510 520 530 540 HTDFIPQHHK DLLPTHSTIA KESVCQAALG LILKEKEMTS AFKLHTQDQF SPFSFSSGRR 550 560 570 580 590 600 LNISYTRNMT LRSGKNDIII AVTYNRDGSY DMQIENKLFR VLGDLSNEDG YTYLKSSVNG 610 620 630 640 650 660 VASKSKFILL DNTIYLFSME GSIEVGIPVP KYLSPVSAEG TQGGTIAPMT GTIEKVFVKA 670 680 690 700 710 GDRVKAGDAL MVMIAMKMEH TIKAPKDGRI KKVFFSEGAQ ANRHAPLVEF EEEEV

References

	« Hide 'large scale' referencesShow 'large scale' references »
[1]	"The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)." The MGC Project Team Genome Res. 14:2121-2127(2004) [PubMed: 15489334] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA]. Strain: Brown Norway. Tissue: Kidney.
[2]	Maurya D.K., Bhargava P. Submitted (JAN-2009) to UniProtKB Cited for: IDENTIFICATION BY MASS SPECTROMETRY.
+	Additional computationally mapped references.

Cross-references

Sequence databases
EMBL GenBank DDBJ	BC088473 mRNA. Translation: AAH88473.1.
IPI	IPI00372191.
RefSeq	NP_001009653.1.
UniGene	Rn.15587
3D structure databases
HSSP	HSSP built from PDB template 1BNC based on UniProtKB P24182.
SMR	Q5I0C3. Positions 45-493, 641-714.
ModBase	Search...
Protein-protein interaction databases
STRING	Q5I0C3.
Genome annotation databases
Ensembl	ENSRNOT00000018942; ENSRNOP00000018942; ENSRNOG00000013293; Rattus norvegicus. [Genome view]
GeneID	294972.
KEGG	rno:294972.
UCSC	NM_001009653. rat.
Organism-specific databases
CTD	294972.
RGD	1310615. Mccc1.
Phylogenomic databases
eggNOG	roNOG07266.
HOVERGEN	Q5I0C3.
InParanoid	Q5I0C3.
Gene expression databases
ArrayExpress	Q5I0C3.
Genevestigator	Q5I0C3.
Family and domain databases
InterPro	IPR011761. ATP-grasp. IPR013816. ATP_grasp_subdomain_2. IPR001882. Biotin_BS. IPR011764. Biotin_carboxylation_dom. IPR005482. Biotin_COase_C. IPR000089. Biotin_lipoyl. IPR005479. CarbamoylP_synth_lsu_ATP-bd. IPR005481. CarbamoylP_synth_lsu_N. IPR013817. Pre-ATP_grasp. IPR016185. PreATP-grasp-like. IPR011054. Rudment_hybrid_motif. IPR011053. Single_hybrid_motif. [Graphical view]
Gene3D	G3DSA:3.30.470.20. ATP_grasp_subdomain_2. 1 hit. G3DSA:3.40.50.20. Pre-ATP_grasp. 1 hit.
Pfam	PF02785. Biotin_carb_C. 1 hit. PF00364. Biotin_lipoyl. 1 hit. PF00289. CPSase_L_chain. 1 hit. PF02786. CPSase_L_D2. 1 hit. [Graphical view]
SMART	SM00878. Biotin_carb_C. 1 hit. [Graphical view]
PROSITE	PS50975. ATP_GRASP. 1 hit. PS50979. BC. 1 hit. PS00188. BIOTIN. 1 hit. PS50968. BIOTINYL_LIPOYL. 1 hit. PS00866. CPSASE_1. False negative. PS00867. CPSASE_2. 1 hit. [Graphical view]
ProtoNet	Search...
Other Resources
NextBio	638797.

Entry information

Entry name

MCCA_RAT

Accession

Primary (citable) accession number: Q5I0C3

Entry history

Integrated into UniProtKB/Swiss-Prot:	March 3, 2009
Last sequence update:	February 15, 2005
Last modified:	January 19, 2010
This is version 48 of the entry and version 1 of the sequence. [Complete history]

Entry status

Reviewed (UniProtKB/Swiss-Prot)

Annotation project

HPI (Human Proteome Initiative)