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    Carnitine Shuttle: DR - Rijha Ahmed

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    Safura Ijaz
    The carnitine shuttle transports long-chain fatty acids from the cytosol into the mitochondrial matrix for beta-oxidation since the inner mitochondrial membrane is impermeable to CoA. Carnitine acts as the carrier in this rate-limiting process. Malonyl CoA inhibits CPT-I to prevent fatty acid entry into mitochondria when fatty acid synthesis is occurring. Liver and kidney can synthesize carnitine while muscle must obtain it from the diet or endogenous synthesis. Deficiencies can decrease the ability to use fatty acids and cause hypoglycemia or exercise intolerance depending on the tissue affected. Treatment involves a high carbohydrate, low fat diet supplemented with medium-chain triglycerides.

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    Carnitine Shuttle: DR - Rijha Ahmed

    Uploaded by

    Safura Ijaz
    22 views12 pages
    The carnitine shuttle transports long-chain fatty acids from the cytosol into the mitochondrial matrix for beta-oxidation since the inner mitochondrial membrane is impermeable to CoA. Carnitine acts as the carrier in this rate-limiting process. Malonyl CoA inhibits CPT-I to prevent fatty acid entry into mitochondria when fatty acid synthesis is occurring. Liver and kidney can synthesize carnitine while muscle must obtain it from the diet or endogenous synthesis. Deficiencies can decrease the ability to use fatty acids and cause hypoglycemia or exercise intolerance depending on the tissue affected. Treatment involves a high carbohydrate, low fat diet supplemented with medium-chain triglycerides.

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    The carnitine shuttle transports long-chain fatty acids from the cytosol into the mitochondrial matrix for beta-oxidation since the inner mitochondrial membrane is impermeable to CoA. Carnitine acts as the carrier in this rate-limiting process. Malonyl CoA inhibits CPT-I to prevent fatty acid entry into mitochondria when fatty acid synthesis is occurring. Liver and kidney can synthesize carnitine while muscle must obtain it from the diet or endogenous synthesis. Deficiencies can decrease the ability to use fatty acids and cause hypoglycemia or exercise intolerance depending on the tissue affected. Treatment involves a high carbohydrate, low fat diet supplemented with medium-chain triglycerides.

    Copyright:

    © All Rights Reserved
    Download as PPTX, PDF, TXT or read online from Scribd
    Download as pptx, pdf, or txt
    22 views12 pages

    Carnitine Shuttle: DR - Rijha Ahmed

    Uploaded by

    Safura Ijaz
    The carnitine shuttle transports long-chain fatty acids from the cytosol into the mitochondrial matrix for beta-oxidation since the inner mitochondrial membrane is impermeable to CoA. Carnitine acts as the carrier in this rate-limiting process. Malonyl CoA inhibits CPT-I to prevent fatty acid entry into mitochondria when fatty acid synthesis is occurring. Liver and kidney can synthesize carnitine while muscle must obtain it from the diet or endogenous synthesis. Deficiencies can decrease the ability to use fatty acids and cause hypoglycemia or exercise intolerance depending on the tissue affected. Treatment involves a high carbohydrate, low fat diet supplemented with medium-chain triglycerides.

    Copyright:

    © All Rights Reserved
    Download as PPTX, PDF, TXT or read online from Scribd
    Download as pptx, pdf, or txt
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    carnitine shuttle

    Dr.Rijha ahmed
    Carnitine shuttle
    • β-oxidation occurs in the mitochondrial matrix,
    • the fatty acid must be transported across the
    inner mitochondrial membrane that is
    impermeable to CoA.
    • Therefore, a specialized carrier transports the
    long-chain acyl group from the cytosol into the
    mitochondrial matrix.
    • This carrier is carnitine, and this rate-limiting
    transport process is called the “carnitine shuttle”
    Inhibitor of the carnitine shuttle
    • Malonyl CoA inhibits CPT-I
    • preventing the entry of long-chain acyl groups into the
    mitochondrial matrix.
    • when fatty acid synthesis is occurring in the cytosol (as indicated by
    the presence of malonyl CoA), the newly made palmitate cannot be
    transferred into mitochondria and degraded. [Note: Muscle,
    although it does not synthesize fatty acids, contains the
    mitochondrial isoform of ACC (ACC2 ), allowing regulation of beta
    oxidation
    • Liver contain both isozymes.
    • Fatty acid oxidation is also regulated by the acetyl CoA to CoA ratio:
    as the ratio increases, the CoA-requiring thiolase reaction decreases
    Sources of carnitine
    • in meat products.
    • Carnitine can also be synthesized from the amino acids lysine
    and methionine by an enzymatic pathway found in the
    • liver and
    • Kidney
    • but not in skeletal or heart muscle.
    • totally dependent on uptake of carnitine provided by
    endogenous synthesis or the diet and distributed by the blood.
    • Skeletal muscle contains about 97% of all carnitine in the
    body.
    Carnitine deficiencies
    • decreased ability of tissues to use LCFAs as a fuel
    • Primary carnitine deficiency: defects in a
    membrane transporter that prevent uptake of
    carnitine by cardiac and skeletal muscle and kidney
    • Secondary carnitine deficiency occurs primarily as
    a result of defects in fatty acid oxidation
     leading to the accumulation of acylcarnitines that
    are excreted in the urine,
     decreasing carnitine availability
    • Acquired secondary carnitine deficiency can
    be seen, for example, in patients with liver
    disease (decreased carnitine synthesis)
    • or those taking the antiseizure drug valproic
    acid (decreased renal reabsorption)
    deficiencies in
    CPT-I
    • CPT-I deficiency affects the liver,
    • where an inability to use LCFAs for fuel greatly
    impairs that tissue’s ability to synthesize
    glucose (an endergonic process) during a fast.
    • This can lead to severe hypoglycemia,
    coma,and death
    CPT-II deficiency
    • CPT-II deficiency can affect the liver and
    cardiac and skeletal muscle.
    • The most common (and least severe) form
    affects skeletal muscle.
    • It presents as muscle weakness with
    myoglobinemia following prolonged exercise
    • When the [NADH]/[NAD] ratio is high, -
    hydroxyacyl-CoA dehydrogenase is inhibited
    • high concentrations of acetyl-CoA inhibit
    thiolase
    Treatment

    • includes avoidance of fasting and


    • adopting a diet high in carbohydrates and low
    in fat but supplemented with medium-chain
    TAGs.

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