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ABS65
Sigma-AldrichAnti-CPT1A Antibody
Detect CPT1A using this Anti-CPT1A Antibody validated for use in Western Blotting.
More>>Detect CPT1A using this Anti-CPT1A Antibody validated for use in Western Blotting. Less<<
Anti-CPT1A Antibody: SDB (Sicherheitsdatenblätter), Analysenzertifikate und Qualitätszertifikate, Dossiers, Broschüren und andere verfügbare Dokumente.
Carnitine palmitoyltransferase 1A (CPT1A, CPT1-L, or L-CPT1) is an hepatic enzyme that, together with CPT2 and carnitine-acylcarnitine translocase, mediates the oxidation of long chain fatty acids to produce energy, in periods of fasting. CPT1A is localized in the outer mitochondrial membrane and catalyzes the addition of carnitine to long-chain fatty acids thereby enabling effective transport of these molecules to the inner mitochondrial membrane where they are metabolized. CPT1A may function as part of a complex consisting of the acyl-CoA synthetase (ACSL) and the voltage-dependent anion channel (VDAC) and is regulated by malonyl-CoA. Deficiencies in CPT1A result in hypoketotic hypoglycemia.
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. [provided by RefSeq, Jul 2008].
TISSUE SPECIFICITY: Strong expression in kidney and heart, and lower in liver and skeletal muscle.
INVOLVEMENT IN DISEASE: Defects in CPT1A are the cause of carnitine palmitoyltransferase 1A deficiency (CPT1AD) [MIM:255120]; also known as CPT-I deficiency or CPT1A deficiency. CPT1AD is a rare autosomal recessive metabolic disorder of long-chain fatty acid oxidation characterized by severe episodes of hypoketotic hypoglycemia usually occurring after fasting or illness. Onset is in infancy or early childhood.
SEQUENCE SIMILARITIESl Belongs to the carnitine/choline acetyltransferase family.
Molecular Weight
~78 kDa observed. An uncharacterized band appears at ~32 kDa in some lysates. The calculated molecular weight is 88 kDa, however CPT1A has been shown as a ~78 kDa band in western blots (Tan, L., et al (2011). J Inherit Metab Dis. 34:443–447.)
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Quality Assurance
Evaluated by Western Blot in mouse liver tissue lysate.
Western Blot Analysis: A 1:1,000 dilution of this antibody detected CPT1A on 10 µg of mouse liver tissue lysate.
Usage Statement
Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
Storage and Shipping Information
Storage Conditions
Stable for 1 year at -20°C from date of receipt. Handling Recommendations: Upon receipt and prior to removing the cap, centrifuge the vial and gently mix the solution. Aliquot into microcentrifuge tubes and store at -20°C. Avoid repeated freeze/thaw cycles, which may damage IgG and affect product performance.
PTC124 improves readthrough and increases enzymatic activity of the CPT1A R160X nonsense mutation. Tan, Lu, et al. J. Inherit. Metab. Dis., 34: 443-7 (2011)
2010
Deficiency of carnitine palmitoyltransferase 1A (CPT1A) results in impaired hepatic long-chain fatty acid oxidation and ketogenesis. We have previously described a patient with a severe CPT1A phenotype who is homozygous for the nonsense mutation 478 C > T (R160X). It has been known for some time that gentamicin can promote readthrough of nonsense codons. Recently, a new compound (PTC124) with less clinical toxicity than gentamicin has been indicated as a therapy for patients with nonsense mutations for multiple genetic diseases. The study is designed to investigate whether PTC124 can promote readthrough of the R160X CPT1A mutation and increase normal sized CPT1 protein expression and activity in the patient's skin fibroblasts. Our study demonstrated that after both PTC 124 and gentamicin treatment, there was an increase in CPT1 activity in patient fibroblasts to levels that are similar to that of the mild Inuit P479L variant. Our results provide additional evidence for proof of principle that PTC124 is a potential therapeutic agent for treating patients with any genetic condition that results from a nonsense mutation.
