Gene/Proteome Database (LMPD)

LMPD ID
LMP000406
Gene ID
5096
Species
Homo sapiens (Human)
Gene Name
propionyl CoA carboxylase, beta polypeptide
Gene Symbol
PCCB
Synonyms
-
Alternate Names
propionyl-CoA carboxylase beta chain, mitochondrial; PCCase subunit beta; propanoyl-CoA:carbon dioxide ligase subunit beta; propionyl Coenzyme A carboxylase, beta polypeptide
Chromosome
3
Map Location
3q21-q22
EC Number
6.4.1.3
Summary
The protein encoded by this gene is a subunit of the propionyl-CoA carboxylase (PCC) enzyme, which is involved in the catabolism of propionyl-CoA. PCC is a mitochondrial enzyme that probably acts as a dodecamer of six alpha subunits and six beta subunits. This gene encodes the beta subunit of PCC. Defects in this gene are a cause of propionic acidemia type II (PA-2). Multiple transcript variants encoding different isoforms have been found for this gene.[provided by RefSeq, May 2010]
Orthologs
View orthologs and multiple alignments for PCCB

Proteins

propionyl-CoA carboxylase beta chain, mitochondrial isoform 1 precursor
Refseq ID NP_000523
Protein GI 119943100
UniProt ID P05166
mRNA ID NM_000532
Length 539
RefSeq Status REVIEWED
MAAALRVAAVGARLSVLASGLRAAVRSLCSQATSVNERIENKRRTALLGGGQRRIDAQHKRGKLTARERISLLLDPGSFVESDMFVEHRCADFGMAADKNKFPGDSVVTGRGRINGRLVYVFSQDFTVFGGSLSGAHAQKICKIMDQAITVGAPVIGLNDSGGARIQEGVESLAGYADIFLRNVTASGVIPQISLIMGPCAGGAVYSPALTDFTFMVKDTSYLFITGPDVVKSVTNEDVTQEELGGAKTHTTMSGVAHRAFENDVDALCNLRDFFNYLPLSSQDPAPVRECHDPSDRLVPELDTIVPLESTKAYNMVDIIHSVVDEREFFEIMPNYAKNIIVGFARMNGRTVGIVGNQPKVASGCLDINSSVKGARFVRFCDAFNIPLITFVDVPGFLPGTAQEYGGIIRHGAKLLYAFAEATVPKVTVITRKAYGGAYDVMSSKHLCGDTNYAWPTAEIAVMGAKGAVEIIFKGHENVEAAQAEYIEKFANPFPAAVRGFVDDIIQPSSTRARICCDLDVLASKKVQRPWRKHANIPL
propionyl-CoA carboxylase beta chain, mitochondrial isoform 2 precursor
Refseq ID NP_001171485
Protein GI 295821216
UniProt ID P05166
mRNA ID NM_001178014
Length 559
RefSeq Status REVIEWED
MAAALRVAAVGARLSVLASGLRAAVRSLCSQATSVNERIENKRRTALLGGGQRRIDAQHKRGKLTARERISLLLDPGSFVESDMFVEHRCADFGMAADKNKFPGDSVVTGRGRINGRLVYVFSQQIIGWAQWLPLVISALWEAEDFTVFGGSLSGAHAQKICKIMDQAITVGAPVIGLNDSGGARIQEGVESLAGYADIFLRNVTASGVIPQISLIMGPCAGGAVYSPALTDFTFMVKDTSYLFITGPDVVKSVTNEDVTQEELGGAKTHTTMSGVAHRAFENDVDALCNLRDFFNYLPLSSQDPAPVRECHDPSDRLVPELDTIVPLESTKAYNMVDIIHSVVDEREFFEIMPNYAKNIIVGFARMNGRTVGIVGNQPKVASGCLDINSSVKGARFVRFCDAFNIPLITFVDVPGFLPGTAQEYGGIIRHGAKLLYAFAEATVPKVTVITRKAYGGAYDVMSSKHLCGDTNYAWPTAEIAVMGAKGAVEIIFKGHENVEAAQAEYIEKFANPFPAAVRGFVDDIIQPSSTRARICCDLDVLASKKVQRPWRKHANIPL

Gene Information

Entrez Gene ID
5096
Gene Name
propionyl CoA carboxylase, beta polypeptide
Gene Symbol
PCCB
Species
Homo sapiens

Gene Ontology (GO Annotations)

GO ID Source Type Description
GO:0005829 TAS:Reactome C cytosol
GO:0005759 TAS:Reactome C mitochondrial matrix
GO:0005739 TAS:ProtInc C mitochondrion
GO:0005524 IEA:UniProtKB-KW F ATP binding
GO:0004658 TAS:ProtInc F propionyl-CoA carboxylase activity
GO:0006768 TAS:Reactome P biotin metabolic process
GO:0044255 TAS:Reactome P cellular lipid metabolic process
GO:0006635 TAS:Reactome P fatty acid beta-oxidation
GO:0019626 TAS:Reactome P short-chain fatty acid catabolic process
GO:0044281 TAS:Reactome P small molecule metabolic process
GO:0006766 TAS:Reactome P vitamin metabolic process
GO:0006767 TAS:Reactome P water-soluble vitamin metabolic process

KEGG Pathway Links

KEGG Pathway ID Description
hsa01200 Carbon metabolism
hsa00630 Glyoxylate and dicarboxylate metabolism

REACTOME Pathway Links

REACTOME Pathway ID Description
REACT_11153 Biotin transport and metabolism
REACT_993 Propionyl-CoA catabolism

Domain Information

InterPro Annotations

Accession Description
IPR011763 Acetyl-coenzyme A carboxyltransferase, C-terminal
IPR011762 Acetyl-coenzyme A carboxyltransferase, N-terminal
IPR000022 Carboxyl transferase
IPR029045 ClpP/crotonase-like domain

UniProt Annotations

Entry Information

Gene Name
propionyl CoA carboxylase, beta polypeptide
Protein Entry
PCCB_HUMAN
UniProt ID
P05166
Species
Human

Comments

Comment Type Description
Alternative Products Event=Alternative splicing; Named isoforms=2; Name=1; IsoId=P05166-1; Sequence=Displayed; Name=2; IsoId=P05166-2; Sequence=VSP_042568; Note=No experimental confirmation available.;
Catalytic Activity ATP + propanoyl-CoA + HCO(3)(-) = ADP + phosphate + (S)-methylmalonyl-CoA.
Disease Propionic acidemia type II (PA-2) [MIM
Pathway Metabolic intermediate metabolism; propanoyl-CoA degradation; succinyl-CoA from propanoyl-CoA
Similarity Belongs to the AccD/PCCB family.
Similarity Contains 1 carboxyltransferase 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 LMP000406 (as displayed in Record Overview)

Protein GI Database Accession Length Protein Name
119943100 RefSeq NP_000523 539 propionyl-CoA carboxylase beta chain, mitochondrial isoform 1 precursor
295821216 RefSeq NP_001171485 559 propionyl-CoA carboxylase beta chain, mitochondrial isoform 2 precursor

Identical Sequences to LMP000406 proteins

Reference Database Accession Length Protein Name
GI:295821216 DBBJ BAH12030.1 559 unnamed protein product [Homo sapiens]
GI:295821216 GenBank EAW79118.1 559 propionyl Coenzyme A carboxylase, beta polypeptide, isoform CRA_c [Homo sapiens]
GI:119943100 GenBank AEQ84553.1 539 Sequence 46 from patent US 8039587
GI:119943100 GenBank AEU53416.1 539 Sequence 46 from patent US 8062891
GI:119943100 GenBank AGO72369.1 539 Sequence 46 from patent US 8470972
GI:119943100 GenBank AGU32360.1 539 Sequence 46 from patent US 8507277
GI:119943100 GenBank AGX48264.1 539 Sequence 46 from patent US 8541550
GI:119943100 GenBank AIC49343.1 539 PCCB, partial [synthetic construct]

Related Sequences to LMP000406 proteins

Reference Database Accession Length Protein Name
GI:119943100 EMBL CAA51825.1 539 propionyl-CoA carboxylase [Homo sapiens]
GI:119943100 EMBL CAH92957.1 539 hypothetical protein [Pongo abelii]
GI:119943100 GenBank AAB28900.1 539 propionyl CoA carboxylase beta subunit [Homo sapiens]
GI:295821216 GenBank AAH13768.1 539 Propionyl Coenzyme A carboxylase, beta polypeptide [Homo sapiens]
GI:295821216 GenBank AAH53661.1 539 Propionyl Coenzyme A carboxylase, beta polypeptide [Homo sapiens]
GI:295821216 GenBank ADZ15494.1 539 propionyl Coenzyme A carboxylase, beta polypeptide, partial [synthetic construct]
GI:119943100 GenBank AED82523.1 539 Sequence 1260 from patent US 7919467
GI:119943100 GenBank AFI35755.1 539 propionyl-CoA carboxylase beta chain, mitochondrial isoform 1 precursor [Macaca mulatta]
GI:295821216 GenBank AGU32360.1 539 Sequence 46 from patent US 8507277
GI:295821216 GenBank AGX48264.1 539 Sequence 46 from patent US 8541550
GI:119943100 RefSeq NP_001126743.1 539 propionyl-CoA carboxylase beta chain, mitochondrial [Pongo abelii]
GI:295821216 SwissProt P05166.3 539 RecName: Full=Propionyl-CoA carboxylase beta chain, mitochondrial; Short=PCCase subunit beta; AltName: Full=Propanoyl-CoA:carbon dioxide ligase subunit beta; Flags: Precursor [Homo sapiens]