pcca-1 Propionyl-CoA carboxylase alpha chain, mitochondrial [Caenorhabditis elegans] - Gene

Summary

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Official Symbol: pcca-1
Official Full Name: Propionyl-CoA carboxylase alpha chain, mitochondrial
Primary source: WormBase:WBGene00017864
Locus tag: CELE_F27D9.5
See related: AllianceGenome:WB:WBGene00017864
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 involved in lipid catabolic process. Located in mitochondrion. Is expressed in body wall musculature; head; intestine; and nervous system. Used to study propionic acidemia. Human ortholog(s) of this gene implicated in amino acid metabolic disorder and propionic acidemia. Orthologous to human PCCA (propionyl-CoA carboxylase subunit alpha). [provided by Alliance of Genome Resources, Dec 2024]
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Genomic context

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See pcca-1 in Genome Data Viewer

Location:: chromosome: X

Exon count:: 7

Sequence:: Chromosome: X; NC_003284.9 (7657542..7660986, complement)

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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Pathways from PubChem

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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

F27D9.5 (e-PCR)
- UniSTS:312306

Homology

Orthologs from OrthoDB

Gene Ontology Provided by WormBase

Function	Evidence Code	Pubs
enables ATP binding	IEA Inferred from Electronic Annotation more info
enables ligase activity	IEA Inferred from Electronic Annotation more info
enables metal ion binding	IEA Inferred from Electronic Annotation more info
enables propionyl-CoA carboxylase activity	IBA Inferred from Biological aspect of Ancestor more info
enables propionyl-CoA carboxylase activity	IEA Inferred from Electronic Annotation more info
enables propionyl-CoA carboxylase activity	ISS Inferred from Sequence or Structural Similarity more info

Process	Evidence Code	Pubs
involved_in lipid catabolic process	IEA Inferred from Electronic Annotation more info

Component	Evidence Code	Pubs
located_in mitochondrial matrix	IEA Inferred from Electronic Annotation more info
located_in mitochondrial matrix	ISS Inferred from Sequence or Structural Similarity more info
located_in mitochondrion	HDA more info	PubMed
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 alpha chain, mitochondrial

NP_509293.1

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

7657542..7660986 complement

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GenBank, FASTA, Sequence Viewer (Graphics)

mRNA and Protein(s)

NM_076892.8 → NP_509293.1 Propionyl-CoA carboxylase alpha chain, mitochondrial [Caenorhabditis elegans]

See identical proteins and their annotated locations for NP_509293.1

Status: REVIEWED

UniProtKB/Swiss-Prot

Q19842

Conserved Domains (5) summary

cd06850
Location:657 → 723

biotinyl_domain; The biotinyl-domain or biotin carboxyl carrier protein (BCCP) domain is present in all biotin-dependent enzymes, such as acetyl-CoA carboxylase, pyruvate carboxylase, propionyl-CoA carboxylase, methylcrotonyl-CoA carboxylase, geranyl-CoA carboxylase, ...

smart00878
Location:383 → 490

Biotin_carb_C; Biotin carboxylase C-terminal domain

COG4770
Location:49 → 724

PccA; Acetyl/propionyl-CoA carboxylase, alpha subunit [Lipid transport and metabolism]

pfam00289
Location:49 → 157

CPSase_L_chain; Carbamoyl-phosphate synthase L chain, N-terminal domain

cl17255
Location:162 → 369

ATP-grasp_4; ATP-grasp domain