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    Urea Cycle: Dr. Amro Yousef Al-Amleh

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    Dr. Amro Yousef
    The urea cycle involves the synthesis of urea from ammonia in the liver. One nitrogen of urea comes from ammonia and the other from aspartate. Glutamate is the precursor of ammonia. The carbon and oxygen of urea are derived from carbon dioxide. The first two reactions occur in mitochondria while the remaining enzymes are located in the cytosol. Hyperammonemia results from liver disease or genetic deficiencies in urea cycle enzymes such as ornithine transcarbamoylase. Amino acids are broken down and their carbon skeletons form intermediates like oxaloacetate, alpha-ketoglutarate, pyruvate, fumarate, and succinyl CoA. The end

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    Urea Cycle: Dr. Amro Yousef Al-Amleh

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    Dr. Amro Yousef
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    The urea cycle involves the synthesis of urea from ammonia in the liver. One nitrogen of urea comes from ammonia and the other from aspartate. Glutamate is the precursor of ammonia. The carbon and oxygen of urea are derived from carbon dioxide. The first two reactions occur in mitochondria while the remaining enzymes are located in the cytosol. Hyperammonemia results from liver disease or genetic deficiencies in urea cycle enzymes such as ornithine transcarbamoylase. Amino acids are broken down and their carbon skeletons form intermediates like oxaloacetate, alpha-ketoglutarate, pyruvate, fumarate, and succinyl CoA. The end

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    The urea cycle involves the synthesis of urea from ammonia in the liver. One nitrogen of urea comes from ammonia and the other from aspartate. Glutamate is the precursor of ammonia. The carbon and oxygen of urea are derived from carbon dioxide. The first two reactions occur in mitochondria while the remaining enzymes are located in the cytosol. Hyperammonemia results from liver disease or genetic deficiencies in urea cycle enzymes such as ornithine transcarbamoylase. Amino acids are broken down and their carbon skeletons form intermediates like oxaloacetate, alpha-ketoglutarate, pyruvate, fumarate, and succinyl CoA. The end

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    10 views45 pages

    Urea Cycle: Dr. Amro Yousef Al-Amleh

    Uploaded by

    Dr. Amro Yousef
    The urea cycle involves the synthesis of urea from ammonia in the liver. One nitrogen of urea comes from ammonia and the other from aspartate. Glutamate is the precursor of ammonia. The carbon and oxygen of urea are derived from carbon dioxide. The first two reactions occur in mitochondria while the remaining enzymes are located in the cytosol. Hyperammonemia results from liver disease or genetic deficiencies in urea cycle enzymes such as ornithine transcarbamoylase. Amino acids are broken down and their carbon skeletons form intermediates like oxaloacetate, alpha-ketoglutarate, pyruvate, fumarate, and succinyl CoA. The end

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    Urea Cycle

    Dr. Amro Yousef Al-Amleh


    Urea Cycle
    • One nitrogen of the urea molecule is supplied by free ammonia, and the
    other nitrogen by aspartate.

    • Glutamate is the immediate precursor of both ammonia.

    •  The carbon and oxygen of urea are derived from CO2.


    • The first two reactions leading to the synthesis of urea occur in the
    mitochondria, whereas the remaining cycle enzymes are located in
    the cytosol.
    1. Carbamoyl phosphate synthetase I (CPS-1)
    2. Ornithine transcarbamoylase (OTC)
    3. Argininosuccinate synthetase
    4. Argininosuccinate synthetase
    5. Arginase
    Overall reaction
    Regulation of Urea cycle
    Fate of Urea
    Sources of ammonia
    Hyperammonemia
     
    • Acquired hyperammonemia  Liver disease is common cause of
    hyperammonemia
    • Congenital hyperammonemia  Genetic deficiencies of each of the
    five enzymes of the urea cycle.
    • Ornithine transcarbamoylase deficiency, which is X-linked, is the most common
    of these disorders.
    • All of the other urea cycle disorders follow autosomal recessive inheritance
    pattern.
    Catabolism of Amino Acids
    Breakdown of the carbon skeletons
    Catabolism of the amino acids
    1. Removal of a-amino groups
    2. Breakdown of the resulting carbon skeletons
    • Oxaloacetate.
    • a-ketoglutarate
    • Fumarate
    • Succinyl CoA
    • Pyruvate
    • Acetyl CoA
    • Acetoacetate
    Amino acids that form oxaloacetate
    1. Aspartae
    2. Asparagine
    Amino acids that form a-ketoglutarate
    1. Glutamate
    2. Glutamine
    3. Arginine
    4. Proline
    5. Histidine
    Glutamate
    Glutamine
    Proline
    Arginine
    Histidine
    Amino acids that form pyruvate
    1. Alanine
    2. Cysteine
    3. Serine
    4. Threonine
    5. Glycine
    6. Tryptophan
    Alanine
    Cysteine
    Serine
    Tryptophan
    Amino acids that form fumarate
    1. Tyrosine
    2. phenylalanine
    Amino acids that form succinyl CoA
    1. Methionine
    2. Valine
    3. Threonine
    4. Isoleucine
    Methionine
    Fate of Homocysteine
    • Homocysteine has two fates:-
    1. Resynthesis of methionine: Homocysteine accepts methyl group from
    N5-methyltetrahydrofolate (N5-methyl-THF) in reaction requiring
    methylcobalamin, coenzyme derived from vitamin B12.
    2. Synthesis of cysteine and production of succinyl CoA
     
    Catabolism of the branched-chain amino acids

    • The branched-chain amino acids, isoleucine, leucine, and valine, are


    essential amino acids.

    • They are metabolized primarily by the peripheral tissues (particularly


    muscle), rather than by the liver.
    1. Transamination  branched-chain amino acid aminotransferase.
    2. Oxidative decarboxylation branched-chain a-keto acid
    dehydrogenase complex
    3. Dehydrogenation FAD-linked dehydrogenation
    4. End products:
    • The catabolism of isoleucine  acetyl CoA and succinyl CoA, rendering it
    both ketogenic and glucogenic.
    • The catabolism of Valine  succinyl CoA and is glucogenic.
    • The catabolism of Leucine,  acetoacetate and acetyl CoA and is ketogenic
    Amino acids that form acetyl CoA or acetoacetyl CoA

    1. Leucine is exclusively ketogenic in its catabolism, forming acetyl CoA


    and acetoacetate.
    2. Isoleucine  acetyl CoA + propionyl CoA.
    3. Lysine: An exclusively ketogenic amino acid. Lysine is ultimately
    converted to acetoacetyl CoA.
    4. Tryptophan  alanine + acetoacetyl CoA.
    5. Phenylalanine acetoacetate + fumarate
    6. Tyrosine  acetoacetate + fumarate
    Oxaloacetate Alpha-KGlu Pyruvate Fumarate Succinyl CoA

    Acetyl CoA or acetoacetate

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