Gene/Proteome Database (LMPD)

LMPD ID
LMP013636
Gene ID
687008
Species
Rattus norvegicus (Rat)
Gene Name
propionyl CoA carboxylase, alpha polypeptide
Gene Symbol
Pcca
Alternate Names
propionyl-CoA carboxylase alpha chain, mitochondrial; PCCase alpha subunit; PCCase subunit alpha; propanoyl-CoA:carbon dioxide ligase alpha subunit; propanoyl-CoA:carbon dioxide ligase subunit alpha; Propionyl Coenzyme A carboxylase alpha polypeptide; propionyl-coenzyme A carboxylase, alpha polypeptide;
Chromosome
15
Map Location
15q25
EC Number
6.4.1.3
Summary
alpha subunit of propionyl coenzyme A carboxylase, which catalyzes the conversion of ATP, propanoyl-CoA, and HCO3- to ADP, phosphate, and (S)-methylmalonyl-CoA [RGD, Feb 2006]
Orthologs
View orthologs and multiple alignments for Pcca

Proteins

propionyl-CoA carboxylase alpha chain, mitochondrial
Refseq ID NP_062203
Protein GI 158303308
UniProt ID P14882
mRNA ID NM_019330
Length 723
MAGLWVRTVALLAARRHWRRSSQQLLWTLKRAPVYSQQCLVVSRSLSSVEYEPKEKTFDKILIANRGEIACRVIKTCRKMGIRTVAIHSDVDASSVHVKMADEAVCVGPAPTSKSYLNMDAIMEAIKKTGAQAVHPGYGFLSENKEFAKCLAAEDVTFIGPDTHAIQAMGDKIESKLLAKRAKVNTIPGFDGVLKDADEAVRIAREIGYPVMIKASAGGGGKGMRIPWDDEETRDGFRFSSQEAASSFGDDRLLIEKFIDNPRHIEIQVLGDKHGNALWLNERECSIQRRNQKVVEEAPSIFLDPETRRAMGEQAVAWPKAVKYSSAGTVEFLVDSQKNFYFLEMNTRLQVEHPVTECITGLDLVQEMILVAKGYPLRHKQEDIPISGWAVECRVYAEDPYKSFGLPSIGRLSQYQEPIHLPGVRVDSGIQPGSDISIYYDPMISKLVTYGSDRAEALKRMEDALDSYVIRGVTHNIPLLREVIINTRFVKGDISTKFLSDVYPDGFKGHMLTPSERDQLLAIASSLFVASQLRAQRFQEHSRVPVIRPDVAKWELSVKLHDEDHTVVASNNGPTFNVEVDGSKLNVTSTWNLASPLLSVNVDGTQRTVQCLSRDAGGNMSIQFLGTVYKVHILTKLAAELNKFMLEKVPKDTSSTLCSPMPGVVVAVSVKPGDMVAEGQEICVIEAMKMQNSMTAGKMGKVKLVHCKAGDTVGEGDLLVELE

Gene Information

Entrez Gene ID
687008
Gene Name
propionyl CoA carboxylase, alpha polypeptide
Gene Symbol
Pcca
Species
Rattus norvegicus

Gene Ontology (GO Annotations)

GO ID Source Type Description
GO:0005739 IDA:RGD C mitochondrion
GO:0005524 IEA:UniProtKB-KW F ATP binding
GO:0004075 IEA:InterPro F biotin carboxylase activity
GO:0046872 IEA:InterPro F metal ion binding
GO:0004658 TAS:RGD F propionyl-CoA carboxylase activity
GO:0009063 TAS:RGD P cellular amino acid catabolic process
GO:0009062 TAS:RGD P fatty acid catabolic process

KEGG Pathway Links

KEGG Pathway ID Description
ko01200 Carbon metabolism
rno01200 Carbon metabolism
ko00630 Glyoxylate and dicarboxylate metabolism
rno00630 Glyoxylate and dicarboxylate metabolism
rno01100 Metabolic pathways
ko00640 Propanoate metabolism
rno00640 Propanoate metabolism
ko00280 Valine, leucine and isoleucine degradation
rno00280 Valine, leucine and isoleucine degradation

BIOCYC Pathway Links

BIOCYC Pathway ID Description
PWY-5130 2-oxobutanoate degradation I
PWY-5328 superpathway of methionine salvage and degradation

Domain Information

InterPro Annotations

Accession Description
IPR011761 ATP-grasp fold
IPR013815 ATP-grasp fold, subdomain 1
IPR013816 ATP-grasp fold, subdomain 2
IPR005482 Biotin carboxylase, C-terminal
IPR011764 Biotin carboxylation domain
IPR001882 Biotin-binding site
IPR000089 Biotin/lipoyl attachment
IPR005481 Carbamoyl-phosphate synthase, large subunit, N-terminal
IPR005479 Carbamoyl-phosphate synthetase large subunit-like, ATP-binding domain
IPR016185 Pre-ATP-grasp domain
IPR011054 Rudiment single hybrid motif
IPR011053 Single hybrid motif

UniProt Annotations

Entry Information

Gene Name
propionyl CoA carboxylase, alpha polypeptide
Protein Entry
PCCA_RAT
UniProt ID
P14882
Species
Rat

Comments

Comment Type Description
Catalytic Activity ATP + propanoyl-CoA + HCO(3)(-) = ADP + phosphate + (S)-methylmalonyl-CoA.
Cofactor Name=biotin; Xref=ChEBI:CHEBI:57586; Note=Biotin.;
Disease Note=Propionic acidemia due to recessively inherited deficiency of PCCase activity often causes life-threatening ketosis and acidosis.
Pathway Metabolic intermediate metabolism; propanoyl-CoA degradation; succinyl-CoA from propanoyl-CoA: step 1/3.
Similarity Contains 1 ATP-grasp domain. {ECO:0000255|PROSITE- ProRule:PRU00409}.
Similarity Contains 1 biotin carboxylation domain
Similarity Contains 1 biotinyl-binding domain
Subcellular Location Mitochondrion matrix.
Subunit Probably a dodecamer composed of six biotin-containing alpha subunits and six beta subunits.

Identical and Related Proteins

Unique RefSeq proteins for LMP013636 (as displayed in Record Overview)

Protein GI Database Accession Length Protein Name
158303308 RefSeq NP_062203 723 propionyl-CoA carboxylase alpha chain, mitochondrial

Identical Sequences to LMP013636 proteins

Reference Database Accession Length Protein Name

Related Sequences to LMP013636 proteins

Reference Database Accession Length Protein Name
GI:158303308 EMBL CBV09183.1 724 unnamed protein product [Mus musculus]
GI:158303308 GenBank AAA88512.1 721 alpha-propionyl-CoA carboxylase (EC 6.4.1.3) [Rattus norvegicus]
GI:158303308 GenBank EDM02587.1 723 rCG36968, isoform CRA_b [Rattus norvegicus]
GI:158303308 SwissProt P14882.3 737 RecName: Full=Propionyl-CoA carboxylase alpha chain, mitochondrial; Short=PCCase subunit alpha; AltName: Full=Propanoyl-CoA:carbon dioxide ligase subunit alpha; Flags: Precursor [Rattus norvegicus]