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ABS65 | Anti-CPT1A Antibody

ABS65
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      Descripción

      Replacement Information

      Ofertas especiales

      Tabla espec. clave

      Species ReactivityKey ApplicationsHostFormatAntibody Type
      R, M WB Rb Serum Polyclonal Antibody
      Description
      Catalogue NumberABS65
      DescriptionAnti-CPT1A Antibody
      Alternate Names
      • Carnitine O-palmitoyltransferase 1, liver isoform
      • CPT1-L
      • Carnitine O-palmitoyltransferase I, liver isoform
      • CPT I
      • CPTI-L
      • Carnitine palmitoyltransferase 1A
      Background InformationCarnitine 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.
      References
      Product Information
      FormatSerum
      Control
      • Mouse liver tissue lysate
      PresentationRabbit polyclonal serum containing 0.05% sodium azide.
      Applications
      ApplicationDetect CPT1A using this Anti-CPT1A Antibody validated for use in Western Blotting.
      Key Applications
      • Western Blotting
      Biological Information
      ImmunogenGST-tagged recombinant protein corresponding to rat CPT1A.
      HostRabbit
      IsotypeIgG
      Species Reactivity
      • Rat
      • Mouse
      Antibody TypePolyclonal Antibody
      Entrez Gene Number
      Entrez Gene SummaryThe 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].
      Gene Symbol
      • CPT1A
      • CPT1
      Purification MethodUnpurified
      UniProt Number
      UniProt SummaryCATALYTIC ACTIVITY: Palmitoyl-CoA + L-carnitine = CoA + L-palmitoylcarnitine.

      ENZYME REGULATION: Inhibitors such as malonyl-CoA interact with its catalytic domain and not with an associated regulatory component.

      PATHWAY: Lipid metabolism; fatty acid beta-oxidation.

      SUBCELLULAR LOCATION: Mitochondrion outer membrane; Multi-pass membrane protein.

      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.)
      Physicochemical Information
      Dimensions
      Materials Information
      Toxicological Information
      Safety Information according to GHS
      Safety Information
      Product Usage Statements
      Quality AssuranceEvaluated 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 ConditionsStable 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.
      Packaging Information
      Material Size100 µL
      Transport Information
      Supplemental Information
      Specifications

      Documentation

      Ficha datos de seguridad (MSDS)

      Título

      Ficha técnica de seguridad del material (MSDS) 

      Certificados de análisis

      CargoNúmero de lote
      Anti-CPT1A 2472960
      Anti-CPT1A - 2262711 2262711
      Anti-CPT1A - 2309300 2309300
      Anti-CPT1A - Q2005655 Q2005655
      Anti-CPT1A -2509119 2509119
      Anti-CPT1A -2768113 2768113
      Anti-CPT1A -2797441 2797441

      Referencias bibliográficas

      Visión general referenciasPub Med ID
      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) 2011

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      21253826 21253826

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