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Branched Chain Amino Acids Metabolism

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76 Questions

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


Which amino acid can produce fumarate based on the information provided?


Among the listed amino acids, which one produces OAA?


What is the major function of valine and isoleucine in the body?

Energy generation and providing precursors to replenish TCA

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


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


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


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

By donating electrons to the electron transport chain

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

Acetyl CoA

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

Transamination reaction

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


Which enzyme deficiency leads to benign cardiovascular and neurological effects?


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

When amino acids are a significant source of metabolic energy

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


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


Which condition results from Fumarylacetoacetate hydrolase deficiency?

Tyrosinemia I

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

Methionine metabolism

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

Black urine due to excretion of alkapton

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

Phenylalanine, tyrosine, and Proline

Which amino acid does not produce glucose during its metabolism?


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


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

Cabbage-like body odor

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


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

Fumarylacetoacetate hydrolase

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


Which amino acid sparingly affects the dietary requirement for niacin?


What is a common symptom of Tyrosinemia II?

Lesions in the eye and skin

What is the fate of leucine during its metabolism?

It produces acetyl CoA and acetoacetate

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

They form succinyl CoA during degradation

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


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?

They contribute to cellular oxidation-reduction reactions

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

Acetyl CoA

What is the role of Cystathionase in amino acid metabolism?

Converts homocysteine to methionine

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

Fumarylacetoacetate hydrolase

Which amino acid cannot produce α-ketoglutarate?


What compound is NOT formed during the degradation of phenylalanine?


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


Which amino acid can be converted to ketone bodies?


What is the main symptom of tyrosinemia I?

Liver failure

Which amino acid produces succinyl CoA?


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

Fumarylacetoacetate synthase

Which amino acid is specifically mentioned for producing fumarate?


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


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

Phosphoserine amino transferase

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


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


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

Growth delay

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?

Cysteine decarboxylase deficiency

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


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

Bile salts

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

Defects in conversion of methionine to cysteine

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

Taurine modifies bile salts like taurocholate

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

Vitamin B12

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

D-amino acid oxidase

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

Glycine Cleavage complex

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


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

DLD gene

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


Which enzyme system is responsible for incorporating proline into collagen?

Prolyl hydroxylase system

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


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


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


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


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

Glutamate-gamma-semi aldehyde (GSA)

Which amino acid can be synthesized from glucose except?


Which amino acid, when degraded, forms histidine?


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


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

Fumarate hydratase

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


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


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


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


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


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


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


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

Glutamine synthetase

This quiz covers the metabolism of branched-chain amino acids, focusing on their role as a universal fuel and the processes involved in their degradation. It also discusses the functions of valine and isoleucine in energy generation and providing precursors for the TCA cycle. Test your knowledge on this essential aspect of biochemistry!

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