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Propionyl Coenzyme A Carboxylase Beta (PCCb)

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Propanoyl-CoA:carbon dioxide ligase subunit beta; Propionyl-CoA carboxylase beta chain, mitochondrial

Propionyl Coenzyme A Carboxylase Beta (PCCb)
CCC D693
HGNC 8654
MGI 1914154
PubMed 12409268
RGD 3265
UniProt P05166
PCCb 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).Propionyl-CoA carboxylase catalyses the carboxylation reaction of propionyl CoA. The product of the reaction is (S)-methylmalonyl CoA. Propionyl CoA is the end product of metabolism of odd-chain fatty acids, and is also a metabolite of most methyl-branched fatty acids. It is also the main metabolite of valine, and together with acetyl-CoA, is a metabolite of isoleucine, as well as a methionine metabolite. Propionyl-CoA is thus of great importance as a glucose precursor.

Organism species: Homo sapiens (Human)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPD693Hu01 Recombinant Propionyl Coenzyme A Carboxylase Beta (PCCb) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies n/a Monoclonal Antibody to Propionyl Coenzyme A Carboxylase Beta (PCCb) Monoclonal Antibody Customized Service Offer
n/a Polyclonal Antibody to Propionyl Coenzyme A Carboxylase Beta (PCCb) Polyclonal Antibody Customized Service Offer
Assay Kits SED693Hu ELISA Kit for Propionyl Coenzyme A Carboxylase Beta (PCCb) Enzyme-linked immunosorbent assay for Antigen Detection.

Organism species: Mus musculus (Mouse)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPD693Mu01 Recombinant Propionyl Coenzyme A Carboxylase Beta (PCCb) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies PAD693Mu01 Polyclonal Antibody to Propionyl Coenzyme A Carboxylase Beta (PCCb) WB; IHC; ICC; IP.
Assay Kits n/a CLIA Kit for Propionyl Coenzyme A Carboxylase Beta (PCCb) CLIA Kit Customized Service Offer
n/a ELISA Kit for Propionyl Coenzyme A Carboxylase Beta (PCCb) ELISA Kit Customized Service Offer

Organism species: Rattus norvegicus (Rat)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins n/a Recombinant Propionyl Coenzyme A Carboxylase Beta (PCCb) Recombinant Protein Customized Service Offer
Antibodies n/a Monoclonal Antibody to Propionyl Coenzyme A Carboxylase Beta (PCCb) Monoclonal Antibody Customized Service Offer
n/a Polyclonal Antibody to Propionyl Coenzyme A Carboxylase Beta (PCCb) Polyclonal Antibody Customized Service Offer
Assay Kits n/a CLIA Kit for Propionyl Coenzyme A Carboxylase Beta (PCCb) CLIA Kit Customized Service Offer
n/a ELISA Kit for Propionyl Coenzyme A Carboxylase Beta (PCCb) ELISA Kit Customized Service Offer
  1. "Correction of the metabolic defect in propionic acidemia fibroblasts by microinjection of a full-length cDNA or RNA transcript encoding the propionyl-CoA carboxylase beta subunit."Genomics 19:500-505(1994) [PubMed] [Europe PMC] [Abstract]
  2. "The molecular defect in propionic acidemia: exon skipping caused by an 8-bp deletion from an intron in the PCCB allele."Hum. Genet. 92:397-402(1993) [PubMed] [Europe PMC] [Abstract]
  3. "Human propionyl-CoA carboxylase beta subunit gene: exon-intron definition and mutation spectrum in Spanish and Latin American propionic acidemia patients."Am. J. Hum. Genet. 63:360-369(1998) [PubMed] [Europe PMC] [Abstract]
  4. "Complete sequencing and characterization of 21,243 full-length human cDNAs." Nat. Genet. 36:40-45(2004) [PubMed] [Europe PMC] [Abstract]
  5. "The DNA sequence, annotation and analysis of human chromosome 3." Nature 440:1194-1198(2006) [PubMed] [Europe PMC] [Abstract]
  6. "The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]
  7. "Isolation of cDNA clones coding for the alpha and beta chains of human propionyl-CoA carboxylase: chromosomal assignments and DNA polymorphisms associated with PCCA and PCCB genes."Proc. Natl. Acad. Sci. U.S.A. 83:4864-4868(1986) [PubMed] [Europe PMC] [Abstract]
  8. "Mitochondrial targeting signals and mature peptides of 3-methylcrotonyl-CoA carboxylase."Biochem. Biophys. Res. Commun. 334:939-946(2005) [PubMed] [Europe PMC] [Abstract]
  9. "An unusual insertion/deletion in the gene encoding the beta-subunit of propionyl-CoA carboxylase is a frequent mutation in Caucasian propionic acidemia."Proc. Natl. Acad. Sci. U.S.A. 87:1372-1376(1990) [PubMed] [Europe PMC] [Abstract]
  10. "Initial characterization of the human central proteome."BMC Syst. Biol. 5:17-17(2011) [PubMed] [Europe PMC] [Abstract]
  11. "An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome."J. Proteomics 96:253-262(2014) [PubMed] [Europe PMC] [Abstract]
  12. "Three independent mutations in the same exon of the PCCB gene: differences between Caucasian and Japanese propionic acidaemia."J. Inherit. Metab. Dis. 16:353-360(1993) [PubMed] [Europe PMC] [Abstract]
  13. "Identification of novel mutations in the PCCB gene in European propionic acidemia patients."Hum. Mutat. 14:89-90(1999) [PubMed] [Europe PMC] [Abstract]
  14. "Overview of mutations in the PCCA and PCCB genes causing propionic acidemia."Hum. Mutat. 14:275-282(1999) [PubMed] [Europe PMC] [Abstract]
  15. "Effect of PCCB gene mutations on the heteromeric and homomeric assembly of propionyl-CoA carboxylase."Mol. Genet. Metab. 74:476-483(2001) [PubMed] [Europe PMC] [Abstract]
  16. "Unexpectedly high prevalence of the mild form of propionic acidemia in Japan: presence of a common mutation and possible clinical implications."Hum. Genet. 111:161-165(2002) [PubMed] [Europe PMC] [Abstract]
  17. "Propionic acidemia: identification of twenty-four novel mutations in Europe and North America."Mol. Genet. Metab. 78:59-67(2003) [PubMed] [Europe PMC] [Abstract]
  18. "Mutation spectrum of the PCCA and PCCB genes in Japanese patients with propionic acidemia."Mol. Genet. Metab. 81:335-342(2004) [PubMed] [Europe PMC] [Abstract]