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 (D)</p> Signup and view all the answers

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

<p>Alanine (A)</p> Signup and view all the answers

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

<p>Methionine (B)</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 (C)</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 (B)</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 (B)</p> Signup and view all the answers

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

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

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

<p>Leucine (A)</p> Signup and view all the answers

Which enzyme deficiency leads to benign cardiovascular and neurological effects?

<p>Cystathionase (D)</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 (A)</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 (B)</p> Signup and view all the answers

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

<p>Homogentisate (B)</p> Signup and view all the answers

Which condition results from Fumarylacetoacetate hydrolase deficiency?

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

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

<p>Methionine metabolism (A)</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 (A)</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 (C)</p> Signup and view all the answers

Which amino acid does not produce glucose during its metabolism?

<p>Leucine (C)</p> Signup and view all the answers

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

<p>Alcaptonuria (A)</p> Signup and view all the answers

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

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

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

<p>Alcaptonuria (B)</p> Signup and view all the answers

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

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

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

<p>Phenylalanine (C)</p> Signup and view all the answers

Which amino acid sparingly affects the dietary requirement for niacin?

<p>Tryptophan (B)</p> Signup and view all the answers

What is a common symptom of Tyrosinemia II?

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

What is the fate of leucine during its metabolism?

<p>It produces acetyl CoA and acetoacetate (D)</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 (B)</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 (A)</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 (B)</p> Signup and view all the answers

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

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

What is the role of Cystathionase in amino acid metabolism?

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

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

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

Which amino acid cannot produce α-ketoglutarate?

<p>Tryptophan (C)</p> Signup and view all the answers

What compound is NOT formed during the degradation of phenylalanine?

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

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

<p>Phenylalanine (A)</p> Signup and view all the answers

Which amino acid can be converted to ketone bodies?

<p>Leucine (A)</p> Signup and view all the answers

What is the main symptom of tyrosinemia I?

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

Which amino acid produces succinyl CoA?

<p>Isoleucine (D)</p> Signup and view all the answers

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

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

Which amino acid is specifically mentioned for producing fumarate?

<p>Tyrosine (A)</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 (A)</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 (A)</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 (C)</p> Signup and view all the answers

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

<p>Serine (D)</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 (D)</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 (D)</p> Signup and view all the answers

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

<p>Cysteine (C)</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 (C)</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 (B)</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 (C)</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 (D)</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 (A)</p> Signup and view all the answers

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

<p>Glycine Cleavage complex (D)</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 (C)</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 (B)</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 (C)</p> Signup and view all the answers

Which enzyme system is responsible for incorporating proline into collagen?

<p>Prolyl hydroxylase system (D)</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 (A)</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 (B)</p> Signup and view all the answers

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

<p>Arginase (D)</p> Signup and view all the answers

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

<p>Ornithine (D)</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) (B)</p> Signup and view all the answers

Which amino acid can be synthesized from glucose except?

<p>Glutamate (A)</p> Signup and view all the answers

Which amino acid, when degraded, forms histidine?

<p>Phenylalanine (B)</p> Signup and view all the answers

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

<p>Glutaminase (A)</p> Signup and view all the answers

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

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

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

<p>NADH (A)</p> Signup and view all the answers

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

<p>Tyrosine (B)</p> Signup and view all the answers

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

<p>Serine (D)</p> Signup and view all the answers

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

<p>Histidine (A)</p> Signup and view all the answers

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

<p>Leucine (A)</p> Signup and view all the answers

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

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

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

<p>Aspartate (B)</p> Signup and view all the answers

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

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

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