GlyCosmos Portal

Submissions

propionyl-CoA carboxylase subunit alpha

Summary

Gene Symbol

PCCA

Organism

Homo sapiens (human)

External Links

NCBI Gene

5095

HGNC

8653

KEGG Gene ID

hsa:5095

PubChem

5095

Alliance of Genome Resources

HGNC:8653

JoGo

PCCA

TogoVar

PCCA

Annotation

Keyword

3D-structure
ATP-binding
Acetylation
Alternative splicing
Biotin
Direct protein sequencing
Disease variant
Ligase
Lipid degradation
Magnesium
Manganese
Metal-binding
Mitochondrion
Phosphoprotein
Proteomics identification
Reference proteome
Transit peptide

Proteins

Displaying 1 entry
UniProt	Protein Name
P05165	Propanoyl-CoA:carbon dioxide ligase subunit alpha

Annotation information from UniProt.

Gene Ontology (GO)

Displaying **all 4** entries
GO Term	Evidence Code	PMID
fatty acid metabolic process	NAS
branched-chain amino acid metabolic process	NAS
short-chain fatty acid catabolic process	IC
succinyl-CoA biosynthetic process	IDA

GO Hierarchy

biological process

cellular process [inference]

metabolic process [inference]

sulfur compound metabolic process [inference]

acyl-CoA metabolic process [inference]

succinyl-CoA metabolic process [inference]

succinyl-CoA biosynthetic process

acyl-CoA biosynthetic process [inference]

succinyl-CoA biosynthetic process

sulfur compound biosynthetic process [inference]

acyl-CoA biosynthetic process [inference]

succinyl-CoA biosynthetic process

phosphorus metabolic process [inference]

phosphate-containing compound metabolic process [inference]

nucleoside phosphate metabolic process [inference]

acyl-CoA metabolic process [inference]

succinyl-CoA metabolic process [inference]

succinyl-CoA biosynthetic process

acyl-CoA biosynthetic process [inference]

succinyl-CoA biosynthetic process

nucleoside phosphate biosynthetic process [inference]

acyl-CoA biosynthetic process [inference]

succinyl-CoA biosynthetic process

organophosphate metabolic process [inference]

nucleoside phosphate metabolic process [inference]

acyl-CoA metabolic process [inference]

succinyl-CoA metabolic process [inference]

succinyl-CoA biosynthetic process

acyl-CoA biosynthetic process [inference]

succinyl-CoA biosynthetic process

nucleoside phosphate biosynthetic process [inference]

acyl-CoA biosynthetic process [inference]

succinyl-CoA biosynthetic process

organophosphate biosynthetic process [inference]

nucleoside phosphate biosynthetic process [inference]

acyl-CoA biosynthetic process [inference]

succinyl-CoA biosynthetic process

catabolic process [inference]

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

biosynthetic process [inference]

nucleobase-containing compound biosynthetic process [inference]

nucleoside phosphate biosynthetic process [inference]

acyl-CoA biosynthetic process [inference]

succinyl-CoA biosynthetic process

sulfur compound biosynthetic process [inference]

acyl-CoA biosynthetic process [inference]

succinyl-CoA biosynthetic process

purine-containing compound biosynthetic process [inference]

acyl-CoA biosynthetic process [inference]

succinyl-CoA biosynthetic process

organophosphate biosynthetic process [inference]

nucleoside phosphate biosynthetic process [inference]

acyl-CoA biosynthetic process [inference]

succinyl-CoA biosynthetic process

primary metabolic process [inference]

nucleobase-containing compound metabolic process [inference]

nucleobase-containing compound biosynthetic process [inference]

nucleoside phosphate biosynthetic process [inference]

acyl-CoA biosynthetic process [inference]

succinyl-CoA biosynthetic process

nucleobase-containing small molecule metabolic process [inference]

nucleoside phosphate metabolic process [inference]

acyl-CoA metabolic process [inference]

succinyl-CoA metabolic process [inference]

succinyl-CoA biosynthetic process

acyl-CoA biosynthetic process [inference]

succinyl-CoA biosynthetic process

nucleoside phosphate biosynthetic process [inference]

acyl-CoA biosynthetic process [inference]

succinyl-CoA biosynthetic process

amino acid metabolic process [inference]

branched-chain amino acid metabolic process

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

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]

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

nucleobase-containing small molecule metabolic process [inference]

nucleoside phosphate metabolic process [inference]

acyl-CoA metabolic process [inference]

succinyl-CoA metabolic process [inference]

succinyl-CoA biosynthetic process

acyl-CoA biosynthetic process [inference]

succinyl-CoA biosynthetic process

nucleoside phosphate biosynthetic process [inference]

acyl-CoA biosynthetic process [inference]

succinyl-CoA biosynthetic process

purine-containing compound metabolic process [inference]

acyl-CoA metabolic process [inference]

succinyl-CoA metabolic process [inference]

succinyl-CoA biosynthetic process

acyl-CoA biosynthetic process [inference]

succinyl-CoA biosynthetic process

purine-containing compound biosynthetic process [inference]

acyl-CoA biosynthetic process [inference]

succinyl-CoA biosynthetic process

Displaying entries **1 - 5** of 10 in total

1

2

Next ›

Last »
GO Term	Evidence Code	PMID
mitochondrion	IDA
mitochondrion	IBA
mitochondrion	HTP
mitochondrial matrix	EXP
mitochondrial matrix	NAS

GO Hierarchy

cellular component

protein-containing complex [inference]

catalytic complex

cellular anatomical structure [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 entries **1 - 5** of 11 in total

1

2

3

Next ›

Last »
GO Term	Evidence Code	PMID
propionyl-CoA carboxylase activity	IDA
propionyl-CoA carboxylase activity	IMP
propionyl-CoA carboxylase activity	IBA
propionyl-CoA carboxylase activity	IEA
protein binding	IPI

GO Hierarchy

molecular function

catalytic activity [inference]

ligase activity [inference]

ligase activity, forming carbon-carbon bonds

CoA carboxylase activity [inference]

propionyl-CoA carboxylase activity

binding [inference]

protein binding

enzyme binding

small molecule binding [inference]

vitamin binding [inference]

biotin binding

ion binding [inference]

anion binding [inference]

carboxylic acid binding [inference]

monocarboxylic acid binding [inference]

biotin binding

purine ribonucleoside triphosphate binding [inference]

ATP binding

cation binding [inference]

metal ion binding

organic acid binding [inference]

carboxylic acid binding [inference]

monocarboxylic acid binding [inference]

biotin binding

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

biotin 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

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

sulfur compound binding [inference]

biotin binding

Gene Ontology information from NCBI Gene and UniProt.

OrthoDB (Group)

Group level

Eukaryota

Group Name

carboxylase

Functional Category

C: Energy production and conversion
E: Amino acid transport and metabolism
I: Lipid transport and metabolism
Q: Secondary metabolites biosynthesis, transport and catabolism

Displaying **all 7** entries
Gene Ontology
ATP binding
catalytic activity
gluconeogenesis
ligase activity
metal ion binding
nucleotide binding
pyruvate carboxylase activity

Displaying **all 10** entries
InterPro
ATP-grasp fold
Biotin carboxylase, C-terminal
Biotin carboxylase-like, N-terminal domain
Biotin carboxylation domain
Biotin-binding site
Biotin/lipoyl attachment
Carbamoyl-phosphate synthetase large subunit-like, ATP-binding domain
Pre-ATP-grasp domain superfamily
Rudiment single hybrid motif
Single hybrid motif

Human Protein Atlas

ENSG00000175198

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

Rhea

RHEA:23720

hydrogencarbonate + ATP + propanoyl-CoA

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

Enzyme

6.4.1.3

PMID

16492739

Disease

Disease Ontology

Displaying **all 2** entries
DO ID	Disease Name	Source
DOID:14701	propionic acidemia	Alliance of Genome Resources
DOID:9252	amino acid metabolic disorder	Alliance of Genome Resources

The Human Phenotype Ontology

Displaying entries **1 - 10** of 40 in total

1

2

3

4

Next ›

Last »
HPO ID	HPO Term
HP:0000007	Autosomal recessive inheritance
HP:0000939	Osteoporosis
HP:0000964	Eczematoid dermatitis
HP:0001249	Intellectual disability
HP:0001250	Seizure
HP:0001254	Lethargy
HP:0001259	Coma
HP:0001263	Global developmental delay
HP:0001332	Dystonia
HP:0001508	Failure to thrive