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propionyl-CoA carboxylase subunit beta

Summary

Gene Symbol

PCCB

Organism

Homo sapiens (human)

External Links

NCBI Gene

5096

HGNC

8654

KEGG Gene ID

hsa:5096

PubChem

5096

Alliance of Genome Resources

HGNC:8654

Annotation

Keyword

3D-structure
ATP-binding
Acetylation
Alternative splicing
Direct protein sequencing
Disease variant
Ligase
Mitochondrion
Phosphoprotein
Reference proteome
Transit peptide

Proteins

Displaying 1 entry
UniProt	Protein Name
P05166	Propanoyl-CoA:carbon dioxide ligase subunit beta

Annotation information from UniProt.

Gene Ontology (GO)

Displaying **all 3** entries
GO Term	Evidence Code	PMID
fatty acid metabolic process		29033250
branched-chain amino acid metabolic process		29033250
short-chain fatty acid catabolic process	IC	6765947

GO Hierarchy

biological process

metabolic process [inference]

nitrogen compound metabolic process [inference]

organonitrogen compound metabolic process [inference]

amino acid metabolic process [inference]

branched-chain amino acid metabolic process

catabolic process [inference]

cellular catabolic process [inference]

organic acid catabolic process [inference]

carboxylic acid catabolic process [inference]

monocarboxylic acid catabolic process [inference]

fatty acid catabolic process [inference]

short-chain fatty acid catabolic process

cellular lipid catabolic process [inference]

fatty acid catabolic process [inference]

short-chain fatty acid catabolic process

small molecule catabolic process [inference]

organic acid catabolic process [inference]

carboxylic acid catabolic process [inference]

monocarboxylic acid catabolic process [inference]

fatty acid catabolic process [inference]

short-chain fatty acid catabolic process

organic substance catabolic process [inference]

lipid catabolic process [inference]

cellular lipid catabolic process [inference]

fatty acid catabolic process [inference]

short-chain fatty acid catabolic process

organic acid catabolic process [inference]

carboxylic acid catabolic process [inference]

monocarboxylic acid catabolic process [inference]

fatty acid catabolic process [inference]

short-chain fatty acid catabolic process

cellular metabolic process [inference]

organic acid metabolic process [inference]

organic acid catabolic process [inference]

carboxylic acid catabolic process [inference]

monocarboxylic acid catabolic process [inference]

fatty acid catabolic process [inference]

short-chain fatty acid catabolic process

oxoacid metabolic process [inference]

carboxylic acid metabolic process [inference]

amino acid metabolic process [inference]

branched-chain amino acid metabolic process

monocarboxylic acid metabolic process [inference]

fatty acid metabolic process

fatty acid catabolic process [inference]

short-chain fatty acid catabolic process

short-chain fatty acid metabolic process [inference]

short-chain fatty acid catabolic process

monocarboxylic acid catabolic process [inference]

fatty acid catabolic process [inference]

short-chain fatty acid catabolic process

carboxylic acid catabolic process [inference]

monocarboxylic acid catabolic process [inference]

fatty acid catabolic process [inference]

short-chain fatty acid catabolic process

cellular catabolic process [inference]

organic acid catabolic process [inference]

carboxylic acid catabolic process [inference]

monocarboxylic acid catabolic process [inference]

fatty acid catabolic process [inference]

short-chain fatty acid catabolic process

cellular lipid catabolic process [inference]

fatty acid catabolic process [inference]

short-chain fatty acid catabolic process

cellular lipid metabolic process [inference]

fatty acid metabolic process

fatty acid catabolic process [inference]

short-chain fatty acid catabolic process

short-chain fatty acid metabolic process [inference]

short-chain fatty acid catabolic process

cellular lipid catabolic process [inference]

fatty acid catabolic process [inference]

short-chain fatty acid catabolic process

primary metabolic process [inference]

amino acid metabolic process [inference]

branched-chain amino acid metabolic process

lipid metabolic process [inference]

lipid catabolic process [inference]

cellular lipid catabolic process [inference]

fatty acid catabolic process [inference]

short-chain fatty acid catabolic process

cellular lipid metabolic process [inference]

fatty acid metabolic process

fatty acid catabolic process [inference]

short-chain fatty acid catabolic process

short-chain fatty acid metabolic process [inference]

short-chain fatty acid catabolic process

cellular lipid catabolic process [inference]

fatty acid catabolic process [inference]

short-chain fatty acid catabolic process

small molecule metabolic process [inference]

organic acid metabolic process [inference]

organic acid catabolic process [inference]

carboxylic acid catabolic process [inference]

monocarboxylic acid catabolic process [inference]

fatty acid catabolic process [inference]

short-chain fatty acid catabolic process

oxoacid metabolic process [inference]

carboxylic acid metabolic process [inference]

amino acid metabolic process [inference]

branched-chain amino acid metabolic process

monocarboxylic acid metabolic process [inference]

fatty acid metabolic process

fatty acid catabolic process [inference]

short-chain fatty acid catabolic process

short-chain fatty acid metabolic process [inference]

short-chain fatty acid catabolic process

monocarboxylic acid catabolic process [inference]

fatty acid catabolic process [inference]

short-chain fatty acid catabolic process

carboxylic acid catabolic process [inference]

monocarboxylic acid catabolic process [inference]

fatty acid catabolic process [inference]

short-chain fatty acid catabolic process

small molecule catabolic process [inference]

organic acid catabolic process [inference]

carboxylic acid catabolic process [inference]

monocarboxylic acid catabolic process [inference]

fatty acid catabolic process [inference]

short-chain fatty acid catabolic process

organic substance metabolic process [inference]

organic acid metabolic process [inference]

organic acid catabolic process [inference]

carboxylic acid catabolic process [inference]

monocarboxylic acid catabolic process [inference]

fatty acid catabolic process [inference]

short-chain fatty acid catabolic process

oxoacid metabolic process [inference]

carboxylic acid metabolic process [inference]

amino acid metabolic process [inference]

branched-chain amino acid metabolic process

monocarboxylic acid metabolic process [inference]

fatty acid metabolic process

fatty acid catabolic process [inference]

short-chain fatty acid catabolic process

short-chain fatty acid metabolic process [inference]

short-chain fatty acid catabolic process

monocarboxylic acid catabolic process [inference]

fatty acid catabolic process [inference]

short-chain fatty acid catabolic process

carboxylic acid catabolic process [inference]

monocarboxylic acid catabolic process [inference]

fatty acid catabolic process [inference]

short-chain fatty acid catabolic process

lipid metabolic process [inference]

lipid catabolic process [inference]

cellular lipid catabolic process [inference]

fatty acid catabolic process [inference]

short-chain fatty acid catabolic process

cellular lipid metabolic process [inference]

fatty acid metabolic process

fatty acid catabolic process [inference]

short-chain fatty acid catabolic process

short-chain fatty acid metabolic process [inference]

short-chain fatty acid catabolic process

cellular lipid catabolic process [inference]

fatty acid catabolic process [inference]

short-chain fatty acid catabolic process

organonitrogen compound metabolic process [inference]

amino acid metabolic process [inference]

branched-chain amino acid metabolic process

organic substance catabolic process [inference]

lipid catabolic process [inference]

cellular lipid catabolic process [inference]

fatty acid catabolic process [inference]

short-chain fatty acid catabolic process

organic acid catabolic process [inference]

carboxylic acid catabolic process [inference]

monocarboxylic acid catabolic process [inference]

fatty acid catabolic process [inference]

short-chain fatty acid catabolic process

cellular process [inference]

cellular metabolic process [inference]

organic acid metabolic process [inference]

organic acid catabolic process [inference]

carboxylic acid catabolic process [inference]

monocarboxylic acid catabolic process [inference]

fatty acid catabolic process [inference]

short-chain fatty acid catabolic process

oxoacid metabolic process [inference]

carboxylic acid metabolic process [inference]

amino acid metabolic process [inference]

branched-chain amino acid metabolic process

monocarboxylic acid metabolic process [inference]

fatty acid metabolic process

fatty acid catabolic process [inference]

short-chain fatty acid catabolic process

short-chain fatty acid metabolic process [inference]

short-chain fatty acid catabolic process

monocarboxylic acid catabolic process [inference]

fatty acid catabolic process [inference]

short-chain fatty acid catabolic process

carboxylic acid catabolic process [inference]

monocarboxylic acid catabolic process [inference]

fatty acid catabolic process [inference]

short-chain fatty acid catabolic process

cellular catabolic process [inference]

organic acid catabolic process [inference]

carboxylic acid catabolic process [inference]

monocarboxylic acid catabolic process [inference]

fatty acid catabolic process [inference]

short-chain fatty acid catabolic process

cellular lipid catabolic process [inference]

fatty acid catabolic process [inference]

short-chain fatty acid catabolic process

cellular lipid metabolic process [inference]

fatty acid metabolic process

fatty acid catabolic process [inference]

short-chain fatty acid catabolic process

short-chain fatty acid metabolic process [inference]

short-chain fatty acid catabolic process

cellular lipid catabolic process [inference]

fatty acid catabolic process [inference]

short-chain fatty acid catabolic process

Displaying **all 4** entries
GO Term	Evidence Code	PMID
cytosol
catalytic complex	IPI	20725044
mitochondrial matrix	IDA NAS TAS	16023992 29033250
mitochondrion	IBA

GO Hierarchy

cellular component

protein-containing complex [inference]

catalytic complex

cellular anatomical entity [inference]

cytosol

membrane-enclosed lumen [inference]

organelle lumen [inference]

intracellular organelle lumen [inference]

mitochondrial matrix

organelle [inference]

membrane-bounded organelle [inference]

intracellular membrane-bounded organelle [inference]

mitochondrion

intracellular organelle [inference]

intracellular membrane-bounded organelle [inference]

mitochondrion

Displaying **all 3** entries
GO Term	Evidence Code	PMID
propionyl-CoA carboxylase activity	IBA IDA	6765947
ATP binding	IEA
protein binding	IPI	20725044 32296183

GO Hierarchy

molecular function

catalytic activity [inference]

ligase activity [inference]

ligase activity, forming carbon-carbon bonds [inference]

CoA carboxylase activity [inference]

propionyl-CoA carboxylase activity

binding [inference]

protein binding

small molecule binding [inference]

heterocyclic compound binding [inference]

nucleotide binding [inference]

purine nucleotide binding [inference]

adenyl nucleotide binding [inference]

adenyl ribonucleotide binding [inference]

ATP binding

purine ribonucleotide binding [inference]

adenyl ribonucleotide binding [inference]

ATP binding

ribonucleotide binding [inference]

purine ribonucleotide binding [inference]

adenyl ribonucleotide binding [inference]

ATP binding

ion binding [inference]

anion binding [inference]

purine ribonucleoside triphosphate binding [inference]

ATP binding

organic cyclic compound binding [inference]

nucleoside phosphate binding [inference]

nucleotide binding [inference]

purine nucleotide binding [inference]

adenyl nucleotide binding [inference]

adenyl ribonucleotide binding [inference]

ATP binding

purine ribonucleotide binding [inference]

adenyl ribonucleotide binding [inference]

ATP binding

ribonucleotide binding [inference]

purine ribonucleotide binding [inference]

adenyl ribonucleotide binding [inference]

ATP binding

purine ribonucleoside triphosphate binding [inference]

ATP binding

carbohydrate derivative binding [inference]

ribonucleotide binding [inference]

purine ribonucleotide binding [inference]

adenyl ribonucleotide binding [inference]

ATP binding

Gene Ontology information from NCBI Gene and UniProt.

Human Protein Atlas

ENSG00000114054

Tissue with high expression from Human Protein Atlas. Tissues that are highly expressed are highlighted.

Data available from v23.0.proteinatlas.org. Image credit: Human Protein Atlas

KEGG BRITE Database

Orthology

K01966

Name

propionyl-CoA carboxylase beta chain [EC:6.4.1.3 2.1.3.15]

References

15890657

Rhea

hydrogencarbonate + ATP + propanoyl-CoA

H⁺ + phosphate + ADP + (S)-methylmalonyl-CoA

Enzyme

6.4.1.3

PMID

16492739

Disease

Disease Ontology

Displaying entries **1 - 10** of 45 in total

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DO ID	Disease Name	Source
DOID:0014667	disease of metabolism	DisGeNET
DOID:0050590	severe congenital neutropenia	DisGeNET
DOID:0050700	cardiomyopathy	DisGeNET
DOID:0060270	pontocerebellar hypoplasia type 2D	DisGeNET
DOID:0080199	colorectal carcinoma	DisGeNET
DOID:0110425	dilated cardiomyopathy 1A	DisGeNET
DOID:1059	intellectual disability	DisGeNET
DOID:11476	osteoporosis	DisGeNET
DOID:11832	visual epilepsy	DisGeNET
DOID:11984	hypertrophic cardiomyopathy	DisGeNET

The Human Phenotype Ontology

Displaying entries **31 - 40** of 40 in total

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HPO ID	HPO Term
HP:0003128	Lactic acidosis
HP:0003353	Propionyl-CoA carboxylase deficiency
HP:0004322	Short stature
HP:0004396	Poor appetite
HP:0006846	Acute encephalopathy
HP:0008872	Feeding difficulties in infancy
HP:0008936	Axial hypotonia
HP:0010978	Abnormality of immune system physiology
HP:0011675	Arrhythmia
HP:0011695	Cerebellar hemorrhage

Displaying **all 2** entries
Disease ID	Disease Name
ORPHA:35	propionic acidemia
OMIM:606054	propionic acidemia

PubChem Disease

GHR Health Conditions

Propionic acidemia

MedGen Diseases

Propionic acidemia

OMIM Phenotypes

PROPIONIC ACIDEMIA

KEGG Diseases

Gene-Disease-Co-Occurrences-in-Literature (PubChem)

LIPID MAPS Gene / Proteome Database

LMPD ID: LMP000406
Gene Name: propionyl CoA carboxylase, beta polypeptide

Ortholog

Displaying entries **1 - 10** of 81 in total

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Species	Gene ID	Alliance of Genome Resources	Orthologous MAtrix
Caenorhabditis elegans	180596	WB:WBGene00018701
Latimeria chalumnae	102364951		LATCH16877
Danio rerio	405861	ZFIN:ZDB-GENE-040426-2467	DANRE29231
Astyanax mexicanus	103029063		ASTMX03195
Ictalurus punctatus	108278028		ICTPU09668
Electrophorus electricus	113576466		ELEEL18573
Salmo salar	106574468		SALSA49520
Salmo salar	106575421		SALSA50478
Salmo trutta	115183341		SALTR50905
Salmo trutta	115196214		SALTR95079

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Acknowledgements

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Partly supported by NIH Common Fund Grant #1U01GM125267-01

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Last updated: August 19, 2024