≡

Carnitine Palmitoyltransferase 1A, Liver (CPT1A)

[Edit]

CPT1-L; Carnitine O-palmitoyltransferase I, liver isoform

Carnitine Palmitoyltransferase 1A, Liver (CPT1A)
CCC F368
HGNC 2328
MGI 1098296
PubMed 14517221
RGD 2396
UniProt P50416
Wiki CPT1A
The mitochondrial oxidation of long-chain fatty acids is initiated by the sequential action of carnitine palmitoyltransferase I (which is located in the outer membrane and is detergent-labile) and carnitine palmitoyltransferase II (which is located in the inner membrane and is detergent-stable), together with a carnitine-acylcarnitine translocase.
CPT I is the key enzyme in the carnitine-dependent transport across the mitochondrial inner membrane and its deficiency results in a decreased rate of fatty acid beta-oxidation. Alternatively spliced transcript variants encoding different isoforms have been found for this gene. Strong expression in kidney and heart, and lower in liver and skeletal muscle.

Organism species: Homo sapiens (Human)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPF368Hu01 Recombinant Carnitine Palmitoyltransferase 1A, Liver (CPT1A) Positive Control; Immunogen; SDS-PAGE; WB.
EPF368Hu61 Eukaryotic Carnitine Palmitoyltransferase 1A, Liver (CPT1A) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies PAF368Hu01 Polyclonal Antibody to Carnitine Palmitoyltransferase 1A, Liver (CPT1A) WB; IHC; ICC; IP.
LAF368Hu71 Biotin-Linked Polyclonal Antibody to Carnitine Palmitoyltransferase 1A, Liver (CPT1A) WB; IHC; ICC.
MAF368Hu21 Monoclonal Antibody to Carnitine Palmitoyltransferase 1A, Liver (CPT1A) WB; IHC; ICC; IP.
LAF368Hu81 FITC-Linked Polyclonal Antibody to Carnitine Palmitoyltransferase 1A, Liver (CPT1A) WB; IHC; ICC; IF.
Assay Kits SEF368Hu ELISA Kit for Carnitine Palmitoyltransferase 1A, Liver (CPT1A) Enzyme-linked immunosorbent assay for Antigen Detection.

Organism species: Mus musculus (Mouse)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPF368Mu01 Recombinant Carnitine Palmitoyltransferase 1A, Liver (CPT1A) Positive Control; Immunogen; SDS-PAGE; WB.
RPF368Mu02 Recombinant Carnitine Palmitoyltransferase 1A, Liver (CPT1A) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies PAF368Mu01 Polyclonal Antibody to Carnitine Palmitoyltransferase 1A, Liver (CPT1A) WB; IHC; ICC; IP.
LAF368Mu81 FITC-Linked Polyclonal Antibody to Carnitine Palmitoyltransferase 1A, Liver (CPT1A) WB; IHC; ICC; IF.
LAF368Mu71 Biotin-Linked Polyclonal Antibody to Carnitine Palmitoyltransferase 1A, Liver (CPT1A) WB; IHC; ICC.
PAF368Mu02 Polyclonal Antibody to Carnitine Palmitoyltransferase 1A, Liver (CPT1A) WB; IHC; ICC; IP.
Assay Kits SEF368Mu ELISA Kit for Carnitine Palmitoyltransferase 1A, Liver (CPT1A) Enzyme-linked immunosorbent assay for Antigen Detection.

Organism species: Rattus norvegicus (Rat)

CATALOG NO. PRODUCT NAME APPLICATIONS
Proteins RPF368Ra01 Recombinant Carnitine Palmitoyltransferase 1A, Liver (CPT1A) Positive Control; Immunogen; SDS-PAGE; WB.
Antibodies PAF368Ra01 Polyclonal Antibody to Carnitine Palmitoyltransferase 1A, Liver (CPT1A) WB; IHC; ICC; IP.
LAF368Ra81 FITC-Linked Polyclonal Antibody to Carnitine Palmitoyltransferase 1A, Liver (CPT1A) WB; IHC; ICC; IF.
LAF368Ra71 Biotin-Linked Polyclonal Antibody to Carnitine Palmitoyltransferase 1A, Liver (CPT1A) WB; IHC; ICC.
MAF368Ra21 Monoclonal Antibody to Carnitine Palmitoyltransferase 1A, Liver (CPT1A) WB; IHC; ICC; IP.
Assay Kits SEF368Ra ELISA Kit for Carnitine Palmitoyltransferase 1A, Liver (CPT1A) Enzyme-linked immunosorbent assay for Antigen Detection.
  1. "Human liver mitochondrial carnitine palmitoyltransferase I: characterization of its cDNA and chromosomal localization and partial analysis of the gene."Proc. Natl. Acad. Sci. U.S.A. 92:1984-1988(1995) [PubMed] [Europe PMC] [Abstract]
  2. "Organization of the human liver carnitine palmitoyltransferase 1 gene (CPT1A) and identification of novel mutations in hypoketotic hypoglycaemia."Hum. Genet. 111:179-189(2002) [PubMed] [Europe PMC] [Abstract]
  3. "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]
  4. "An intronic peroxisome proliferator-activated receptor-binding sequence mediates fatty acid induction of the human carnitine palmitoyltransferase 1A."J. Mol. Biol. 354:751-759(2005) [PubMed] [Europe PMC] [Abstract]
  5. "Initial characterization of the human central proteome."BMC Syst. Biol. 5:17-17(2011) [PubMed] [Europe PMC] [Abstract]
  6. "Structural model of carnitine palmitoyltransferase I based on the carnitine acetyltransferase crystal."Biochem. J. 379:777-784(2004) [PubMed] [Europe PMC] [Abstract]
  7. "An environment-dependent structural switch underlies the regulation of carnitine palmitoyltransferase 1A."J. Biol. Chem. 286:42545-42554(2011) [PubMed] [Europe PMC] [Abstract]
  8. "Molecular basis of hepatic carnitine palmitoyltransferase I deficiency."J. Clin. Invest. 102:527-531(1998) [PubMed] [Europe PMC] [Abstract]
  9. "Molecular characterization of L-CPT I deficiency in six patients: insights into function of the native enzyme."J. Lipid Res. 42:1134-1142(2001) [PubMed] [Europe PMC] [Abstract]
  10. "Molecular and enzymatic characterization of a unique carnitine palmitoyltransferase 1A mutation in the Hutterite community."Mol. Genet. Metab. 73:46-54(2001) [PubMed] [Europe PMC] [Abstract]
  11. "Expression analysis of two mutations in carnitine palmitoyltransferase IA deficiency."J. Hum. Genet. 47:342-347(2002) [PubMed] [Europe PMC] [Abstract]
  12. "Functional and structural basis of carnitine palmitoyltransferase 1A deficiency."J. Biol. Chem. 278:50428-50434(2003) [PubMed] [Europe PMC] [Abstract]
  13. "Successful long-term treatment of hepatic carnitine palmitoyltransferase I deficiency and a novel mutation."J. Inherit. Metab. Dis. 27:679-684(2004) [PubMed] [Europe PMC] [Abstract]
  14. "Novel mutations in CPT 1A define molecular heterogeneity of hepatic carnitine palmitoyltransferase I deficiency."Mol. Genet. Metab. 82:59-63(2004) [PubMed] [Europe PMC] [Abstract]