Branched Chain Amino Acids Metabolism
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Questions and Answers

Which amino acid can produce acetyl-CoA directly according to the text?

  • Leucine (correct)
  • Methionine
  • Valine
  • Threonine
  • Which amino acid can produce fumarate based on the information provided?

  • Serine
  • Cysteine
  • Phenylalanine (correct)
  • Tyrosine
  • Among the listed amino acids, which one produces OAA?

  • Asparagine (correct)
  • Histidine
  • Glycine
  • Tryptophan
  • What is the major function of valine and isoleucine in the body?

    <p>Energy generation and providing precursors to replenish TCA</p> Signup and view all the answers

    Which amino acid is specifically mentioned for its degradation in extrahepatic tissues?

    <p>Alanine</p> Signup and view all the answers

    Which amino acid can produce succinyl CoA according to the text?

    <p>Methionine</p> Signup and view all the answers

    Which of the following tissues does not have the branched chain α-keto acid dehydrogenase complex?

    <p>Liver</p> Signup and view all the answers

    How do NADH and FAD(2H) contribute to energy production in the degradation of branched-chain amino acids?

    <p>By donating electrons to the electron transport chain</p> Signup and view all the answers

    In the degradation of isoleucine, what compound does it also form apart from succinyl CoA?

    <p>Acetyl CoA</p> Signup and view all the answers

    What is the first step in the degradation of branched-chain amino acids?

    <p>Transamination reaction</p> Signup and view all the answers

    Which branched-chain amino acid does not produce succinyl CoA during its metabolism?

    <p>Leucine</p> Signup and view all the answers

    Which enzyme deficiency leads to benign cardiovascular and neurological effects?

    <p>Cystathionase</p> Signup and view all the answers

    Under what conditions can amino acid degradation be critical to survival?

    <p>When amino acids are a significant source of metabolic energy</p> Signup and view all the answers

    Which compound is NOT an intermediate through which carbon skeletons of amino acids enter the TCA cycle?

    <p>Malonyl-CoA</p> Signup and view all the answers

    Which enzyme deficiency results in mental retardation, black urine, and arthritis?

    <p>Homogentisate</p> Signup and view all the answers

    Which condition results from Fumarylacetoacetate hydrolase deficiency?

    <p>Tyrosinemia I</p> Signup and view all the answers

    Which amino acid metabolism pathway involves the enzyme Cystathionine β-synthase?

    <p>Methionine metabolism</p> Signup and view all the answers

    What is the consequence of the accumulation of homogentisate acid in blood and tissues?

    <p>Black urine due to excretion of alkapton</p> Signup and view all the answers

    Which amino acids are categorized as ketogenic and can produce acetyl CoA or acetoacetate?

    <p>Phenylalanine, tyrosine, and Proline</p> Signup and view all the answers

    Which amino acid does not produce glucose during its metabolism?

    <p>Leucine</p> Signup and view all the answers

    In which condition would you observe the excretion of alkapton in urine?

    <p>Alcaptonuria</p> Signup and view all the answers

    What is a characteristic symptom of Tyrosinemia I (tyrosinosis)?

    <p>Cabbage-like body odor</p> Signup and view all the answers

    Which disorder is likely to present with a symptom of black urine?

    <p>Alcaptonuria</p> Signup and view all the answers

    What enzyme deficiency leads to death within the first year in affected individuals?

    <p>Fumarylacetoacetate hydrolase</p> Signup and view all the answers

    Which amino acid change leads to brain and developmental damage due to its accumulation?

    <p>Phenylalanine</p> Signup and view all the answers

    Which amino acid sparingly affects the dietary requirement for niacin?

    <p>Tryptophan</p> Signup and view all the answers

    What is a common symptom of Tyrosinemia II?

    <p>Lesions in the eye and skin</p> Signup and view all the answers

    What is the fate of leucine during its metabolism?

    <p>It produces acetyl CoA and acetoacetate</p> Signup and view all the answers

    Which of the following is true regarding the branched-chain amino acids Isoleucine and Valine

    <p>They form succinyl CoA during degradation</p> Signup and view all the answers

    In the degradation of which amino acid does the defect in branched chain alpha-keto acid dehydrogenase lead to Maple syrup urine disease?

    <p>Leucine</p> Signup and view all the answers

    Which of the following statements accurately reflects the role of NADH and FAD(2H) in the degradation of branched-chain amino acids as per the text?

    <p>They contribute to cellular oxidation-reduction reactions</p> Signup and view all the answers

    Which compound is formed by isoleucine in addition to succinyl CoA during its degradation?

    <p>Acetyl CoA</p> Signup and view all the answers

    What is the role of Cystathionase in amino acid metabolism?

    <p>Converts homocysteine to methionine</p> Signup and view all the answers

    Which enzyme deficiency results in liver failure, death, and neurological damage?

    <p>Fumarylacetoacetate hydrolase</p> Signup and view all the answers

    Which amino acid cannot produce α-ketoglutarate?

    <p>Tryptophan</p> Signup and view all the answers

    What compound is NOT formed during the degradation of phenylalanine?

    <p>malonyl-CoA</p> Signup and view all the answers

    Which amino acid can generate acetyl-CoA via acetoacetyl-CoA?

    <p>Phenylalanine</p> Signup and view all the answers

    Which amino acid can be converted to ketone bodies?

    <p>Leucine</p> Signup and view all the answers

    What is the main symptom of tyrosinemia I?

    <p>Liver failure</p> Signup and view all the answers

    Which amino acid produces succinyl CoA?

    <p>Isoleucine</p> Signup and view all the answers

    Which enzyme is responsible for converting tyrosine to Fumarylacetoacetate in amino acid metabolism?

    <p>Fumarylacetoacetate synthase</p> Signup and view all the answers

    Which amino acid is specifically mentioned for producing fumarate?

    <p>Tyrosine</p> Signup and view all the answers

    Which amino acid is synthesized from intermediates of glycolysis and is involved in one-carbon metabolism?

    <p>Serine</p> Signup and view all the answers

    In Smith-Magenis Syndrome, what enzyme deficiency leads to the lack of methyl group donation to THF?

    <p>Phosphoserine amino transferase</p> Signup and view all the answers

    Which amino acid is strictly ketogenic and reconverted to TCA cycle intermediates during degradation as per the text?

    <p>Leucine</p> Signup and view all the answers

    Which intermediate derived from glycolysis donates a methyl group to tetrahydrofolate in one-carbon metabolism?

    <p>Serine</p> Signup and view all the answers

    What is the major symptom associated with deletion 17p11.2 resulting in the lack of an enzyme and Smith-Magenis Syndrome?

    <p>Growth delay</p> Signup and view all the answers

    Which enzyme deficiency is associated with defects in the cystathionine Beta-synthase (CBS) gene and leads to elevated levels of homocysteine and homocystine in blood?

    <p>Cysteine decarboxylase deficiency</p> Signup and view all the answers

    Which amino acid is a source for coenzyme-A synthesis as mentioned in the text?

    <p>Cysteine</p> Signup and view all the answers

    Which sulfur-containing molecule is activated to form PAPS (3'-phosphoadenosine 5'-phosphosulfate) for donating sulfate in modifying carbohydrates and proteins?

    <p>Bile salts</p> Signup and view all the answers

    What is the most common cause of homocystinuria resulting from the malfunction of cystathionine Beta-synthase (CBS)?

    <p>Defects in conversion of methionine to cysteine</p> Signup and view all the answers

    What is the primary function of taurine in the context of sulfur transferase reactions according to the text?

    <p>Taurine modifies bile salts like taurocholate</p> Signup and view all the answers

    What vitamin is required for the synthesis of cysteine from the essential amino acid methionine?

    <p>Vitamin B12</p> Signup and view all the answers

    Which enzyme is responsible for the conversion of glycine to glyoxylate in a medically important degradative pathway?

    <p>D-amino acid oxidase</p> Signup and view all the answers

    What complex, requiring tetrahydrofolate, is the T subunit of glycine dehydrogenase associated with?

    <p>Glycine Cleavage complex</p> Signup and view all the answers

    In the catabolism of glycine, what compound can lead to kidney stone formation when it precipitates in kidney tubules?

    <p>Oxalate</p> Signup and view all the answers

    Which gene at 7q31-q32 encodes the Dihydrolipoamide DH enzyme required for various metabolic complexes?

    <p>DLD gene</p> Signup and view all the answers

    What amino acid is converted to serine by the enzyme serine hydroxymethyltransferase in a key step of its metabolism?

    <p>Glycine</p> Signup and view all the answers

    Which enzyme system is responsible for incorporating proline into collagen?

    <p>Prolyl hydroxylase system</p> Signup and view all the answers

    What makes up 4% of all amino acids in animal tissues due to its importance in collagen stability?

    <p>Hydroxyproline</p> Signup and view all the answers

    Which amino acid is synthesized from glutamate via glutamate semialdehyde on its way to forming ornithine?

    <p>Arginine</p> Signup and view all the answers

    Which enzyme is involved in the degradation of arginine and ornithine?

    <p>Arginase</p> Signup and view all the answers

    Which amino acid is an intermediate of the urea cycle and is synthesized from glutamate?

    <p>Ornithine</p> Signup and view all the answers

    Which compound serves as a link between arginine and glutamate in amino acid metabolism pathways?

    <p>Glutamate-gamma-semi aldehyde (GSA)</p> Signup and view all the answers

    Which amino acid can be synthesized from glucose except?

    <p>Glutamate</p> Signup and view all the answers

    Which amino acid, when degraded, forms histidine?

    <p>Phenylalanine</p> Signup and view all the answers

    Which enzyme is responsible for the conversion of glutamine back to glutamate in the kidney?

    <p>Glutaminase</p> Signup and view all the answers

    What enzyme deficiency has been linked to cancers like leiomyoma and renal cysts?

    <p>Fumarate hydratase</p> Signup and view all the answers

    Which molecule is NOT involved in the conversion of glutamate to alpha-ketoglutarate?

    <p>NADH</p> Signup and view all the answers

    From which amino acid can fumarate be formed in the urea cycle?

    <p>Tyrosine</p> Signup and view all the answers

    Which amino acid is produced from alpha-ketoglutarate by transamination or the glutamate dehydrogenase reaction?

    <p>Serine</p> Signup and view all the answers

    Which amino acid can generate malate for transport into mitochondria from cytosolic fumarate?

    <p>Histidine</p> Signup and view all the answers

    Which amino acid can be reconverted to alpha-ketoglutarate when degraded?

    <p>Leucine</p> Signup and view all the answers

    What nitrogen-providing molecule helps in the formation of asparagine from aspartate?

    <p>NH4+</p> Signup and view all the answers

    Which amino acid is directly produced by the transamination of oxaloacetate?

    <p>Aspartate</p> Signup and view all the answers

    What is the key enzyme responsible for converting glutamine to glutamate?

    <p>Glutamine synthetase</p> Signup and view all the answers

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