pccb-1 Propionyl-CoA carboxylase beta chain, mitochondrial [Caenorhabditis elegans] - Gene

Summary

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Official Symbol: pccb-1
Official Full Name: Propionyl-CoA carboxylase beta chain, mitochondrial
Primary source: WormBase:WBGene00018701
Locus tag: CELE_F52E4.1
See related: AllianceGenome:WB:WBGene00018701
Gene type: protein coding
RefSeq status: REVIEWED
Organism: Caenorhabditis elegans (strain: Bristol N2)
Lineage: Eukaryota; Metazoa; Ecdysozoa; Nematoda; Chromadorea; Rhabditida; Rhabditina; Rhabditomorpha; Rhabditoidea; Rhabditidae; Peloderinae; Caenorhabditis
Summary: Predicted to enable propionyl-CoA carboxylase activity. Predicted to be located in mitochondrial matrix. Predicted to be active in mitochondrion. Human ortholog(s) of this gene implicated in amino acid metabolic disorder and propionic acidemia. Orthologous to human PCCB (propionyl-CoA carboxylase subunit beta). [provided by Alliance of Genome Resources, Jan 2025]
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Genomic context

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See pccb-1 in Genome Data Viewer

Location:: chromosome: X

Exon count:: 5

Sequence:: Chromosome: X; NC_003284.9 (3089549..3092437)

Chromosome X - NC_003284.9 Genomic Context describing neighboring genes

Genomic regions, transcripts, and products

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Go to reference sequence details

Genomic Sequence:: NC_003284.9

Go to nucleotide: Graphics FASTA GenBank

Bibliography

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GeneRIFs: Gene References Into Functions

What's a GeneRIF?

Two C. elegans model animals of propionic acidemia with single-gene pcca-1 or pccb-1 deletions have reduced lifespan with significantly reduced mitochondrial energy metabolism and increased oxidative stress
Title: Propionyl-CoA carboxylase pcca-1 and pccb-1 gene deletions in Caenorhabditis elegans globally impair mitochondrial energy metabolism.

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Interactions

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Products	Interactant	Other Gene	Complex	Source	Pubs	Description

General gene information

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Markers

F52E4.1 (e-PCR)
- UniSTS:311721

Homology

Orthologs from OrthoDB

Gene Ontology Provided by WormBase

Function	Evidence Code	Pubs
enables propionyl-CoA carboxylase activity	IBA Inferred from Biological aspect of Ancestor more info

Component	Evidence Code	Pubs
located_in mitochondrial matrix	IEA Inferred from Electronic Annotation more info
is_active_in mitochondrion	IBA Inferred from Biological aspect of Ancestor more info
located_in mitochondrion	IEA Inferred from Electronic Annotation more info

General protein information

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Preferred Names: Propionyl-CoA carboxylase beta chain, mitochondrial

NP_741742.1

Confirmed by transcript evidence

NP_741743.1

Confirmed by transcript evidence

NCBI Reference Sequences (RefSeq)

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Genome Annotation

The following sections contain reference sequences that belong to a specific genome build. Explain

Reference assembly

Genomic

NC_003284.9 Reference assembly

Range

3089549..3092437

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GenBank, FASTA, Sequence Viewer (Graphics)

mRNA and Protein(s)

NM_171647.8 → NP_741742.1 Propionyl-CoA carboxylase beta chain, mitochondrial [Caenorhabditis elegans]

See identical proteins and their annotated locations for NP_741742.1

Status: REVIEWED

UniProtKB/TrEMBL

Q8WQF1

Conserved Domains (2) summary

COG4799
Location:9 → 522

MmdA; Acetyl-CoA carboxylase, carboxyltransferase component [Lipid transport and metabolism]

cl23717
Location:48 → 250

crotonase-like; Crotonase/Enoyl-Coenzyme A (CoA) hydratase superfamily. This superfamily contains a diverse set of enzymes including enoyl-CoA hydratase, napthoate synthase, methylmalonyl-CoA decarboxylase, 3-hydoxybutyryl-CoA dehydratase, and dienoyl-CoA isomerase. ...
NM_171648.5 → NP_741743.1 Propionyl-CoA carboxylase beta chain, mitochondrial [Caenorhabditis elegans]

See identical proteins and their annotated locations for NP_741743.1

Status: REVIEWED

UniProtKB/TrEMBL

Q20676

Conserved Domains (2) summary

COG4799
Location:23 → 536

MmdA; Acetyl-CoA carboxylase, carboxyltransferase component [Lipid transport and metabolism]

cl23717
Location:62 → 264

crotonase-like; Crotonase/Enoyl-Coenzyme A (CoA) hydratase superfamily. This superfamily contains a diverse set of enzymes including enoyl-CoA hydratase, napthoate synthase, methylmalonyl-CoA decarboxylase, 3-hydoxybutyryl-CoA dehydratase, and dienoyl-CoA isomerase. ...