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In a child with \(BH_4\) deficiency, which enzyme is directly affected by low serotonin levels?
Tryptophan hydroxylase.
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What cofactor is required to convert tryptophan to 5-hydroxytryptophan?
\(BH_4\)
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Which vitamin deficiency impairs the conversion of 5-HTP to serotonin?
Vitamin \(B_6\) (PLP).
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Which enzyme is responsible for the degradation of serotonin?
MAO-A.
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Which neurotransmitters are increased during MAOI therapy?
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Pellagra-like symptoms resulting from defective tryptophan metabolism are linked to the deficiency of which vitamin?
Vitamin \(B_6\).
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Which enzyme is responsible for converting histidine into histamine?
Histidine decarboxylase.
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What cofactor is necessary for the activity of histidine decarboxylase?
PLP (Vitamin \(B_6\)).
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Which enzyme is responsible for synthesizing GABA from glutamate?
Glutamate decarboxylase.
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A Vitamin B6-deficient infant develops seizures due to a decrease in which neurotransmitter?
GABA.
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What is the rate-limiting enzyme in heme synthesis?
\(\delta\)-ALA synthase.
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Which cofactor is required for the activity of ALA synthase?
PLP.
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Name the amino acid precursors for creatine synthesis.
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What methyl donor is used to convert guanidinoacetate to creatine?
SAM.
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Which enzyme catalyzes the conversion between creatine and phosphocreatine?
Creatine kinase.
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Which enzyme converts arginine into urea and ornithine?
Arginase.
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Which enzyme synthesizes nitric oxide?
Nitric oxide synthase (NOS).
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NOS converts arginine into which two products?
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Which amino acids are branched-chain amino acids (BCAAs)?
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What is the first step of BCAA metabolism?
Transamination.
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Which enzyme catalyzes BCAA transamination?
Branched-chain aminotransferase (BCAT).
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Which cofactor does BCAT require?
PLP.
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Which enzyme is deficient in Maple Syrup Urine Disease (MSUD)?
Branched-chain α-ketoacid dehydrogenase (BCKD).
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What are the five cofactors required by BCKD?
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Which BCAA is primarily responsible for neurotoxicity in MSUD?
Leucine.
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Which metabolite is responsible for the maple syrup odor of urine in Maple Syrup Urine Disease?
Isoleucine metabolites.
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What is the diagnosis for a neonate presenting with poor feeding, seizures, and sweet-smelling urine?
Maple Syrup Urine Disease.
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Which enzyme is responsible for converting homocysteine into cystathionine?
Cystathionine β-synthase (CBS).
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Which cofactor is required for the activity of cystathionine β-synthase (CBS)?
PLP.
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Which enzyme catalyzes the remethylation of homocysteine to form methionine?
Methionine synthase.
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What cofactors are required for the function of methionine synthase?
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Which molecule serves as the universal methyl donor?
SAM.
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Which enzyme is responsible for the synthesis of SAM?
Methionine adenosyltransferase.
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A deficiency in which enzyme causes classical homocystinuria?
CBS.
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What is the diagnosis for a patient presenting with lens dislocation, marfanoid habitus, thrombosis, \(\uparrow\) methionine, and \(\uparrow\) homocysteine?
Classical homocystinuria.
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Why is dietary methionine restriction required in patients with classical homocystinuria?
Methionine is converted into homocysteine.
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Why does cysteine become an essential amino acid in cases of cystathionine beta-synthase (CBS) deficiency?
It cannot be synthesized from homocysteine.
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Name the amino acids that generate propionyl-CoA.
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What type of fatty acids are known to generate propionyl-CoA?
Odd-chain fatty acids.
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Which enzyme is responsible for the conversion of propionyl-CoA to methylmalonyl-CoA?
Propionyl-CoA carboxylase.
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Which cofactor is required for the function of propionyl-CoA carboxylase?
Biotin (Vitamin B7).
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Which enzyme converts methylmalonyl-CoA into succinyl-CoA?
Methylmalonyl-CoA mutase.
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What cofactor is required by methylmalonyl-CoA mutase?
Adenosylcobalamin (Vitamin B12).
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What is the diagnosis for a neonate presenting with metabolic acidosis and hyperammonemia resulting from impaired propionyl-CoA to methylmalonyl-CoA conversion?
Propionic acidemia.
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What deficiency should be suspected in a patient with elevated methylmalonic acid and neurologic deterioration?
Methylmalonyl-CoA mutase or Vitamin B12 deficiency.
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In a child with \(BH_4\) deficiency, which enzyme is directly affected by low serotonin levels?
Tryptophan hydroxylase.
What cofactor is required to convert tryptophan to 5-hydroxytryptophan?
\(BH_4\)
Which vitamin deficiency impairs the conversion of 5-HTP to serotonin?
Vitamin \(B_6\) (PLP).
Which enzyme is responsible for the degradation of serotonin?
MAO-A.
Which neurotransmitters are increased during MAOI therapy?
Pellagra-like symptoms resulting from defective tryptophan metabolism are linked to the deficiency of which vitamin?
Vitamin \(B_6\).
Which enzyme is responsible for converting histidine into histamine?
Histidine decarboxylase.
What cofactor is necessary for the activity of histidine decarboxylase?
PLP (Vitamin \(B_6\)).
Which enzyme is responsible for synthesizing GABA from glutamate?
Glutamate decarboxylase.
A Vitamin B6-deficient infant develops seizures due to a decrease in which neurotransmitter?
GABA.
What is the rate-limiting enzyme in heme synthesis?
\(\delta\)-ALA synthase.
Which cofactor is required for the activity of ALA synthase?
PLP.
Name the amino acid precursors for creatine synthesis.
What methyl donor is used to convert guanidinoacetate to creatine?
SAM.
Which enzyme catalyzes the conversion between creatine and phosphocreatine?
Creatine kinase.
Which enzyme converts arginine into urea and ornithine?
Arginase.
Which enzyme synthesizes nitric oxide?
Nitric oxide synthase (NOS).
NOS converts arginine into which two products?
Which amino acids are branched-chain amino acids (BCAAs)?
What is the first step of BCAA metabolism?
Transamination.
Which enzyme catalyzes BCAA transamination?
Branched-chain aminotransferase (BCAT).
Which cofactor does BCAT require?
PLP.
Which enzyme is deficient in Maple Syrup Urine Disease (MSUD)?
Branched-chain α-ketoacid dehydrogenase (BCKD).
What are the five cofactors required by BCKD?
Which BCAA is primarily responsible for neurotoxicity in MSUD?
Leucine.
Which metabolite is responsible for the maple syrup odor of urine in Maple Syrup Urine Disease?
Isoleucine metabolites.
What is the diagnosis for a neonate presenting with poor feeding, seizures, and sweet-smelling urine?
Maple Syrup Urine Disease.
Which enzyme is responsible for converting homocysteine into cystathionine?
Cystathionine β-synthase (CBS).
Which cofactor is required for the activity of cystathionine β-synthase (CBS)?
PLP.
Which enzyme catalyzes the remethylation of homocysteine to form methionine?
Methionine synthase.
What cofactors are required for the function of methionine synthase?
Which molecule serves as the universal methyl donor?
SAM.
Which enzyme is responsible for the synthesis of SAM?
Methionine adenosyltransferase.
A deficiency in which enzyme causes classical homocystinuria?
CBS.
What is the diagnosis for a patient presenting with lens dislocation, marfanoid habitus, thrombosis, \(\uparrow\) methionine, and \(\uparrow\) homocysteine?
Classical homocystinuria.
Why is dietary methionine restriction required in patients with classical homocystinuria?
Methionine is converted into homocysteine.
Why does cysteine become an essential amino acid in cases of cystathionine beta-synthase (CBS) deficiency?
It cannot be synthesized from homocysteine.
Name the amino acids that generate propionyl-CoA.
What type of fatty acids are known to generate propionyl-CoA?
Odd-chain fatty acids.
Which enzyme is responsible for the conversion of propionyl-CoA to methylmalonyl-CoA?
Propionyl-CoA carboxylase.
Which cofactor is required for the function of propionyl-CoA carboxylase?
Biotin (Vitamin B7).
Which enzyme converts methylmalonyl-CoA into succinyl-CoA?
Methylmalonyl-CoA mutase.
What cofactor is required by methylmalonyl-CoA mutase?
Adenosylcobalamin (Vitamin B12).
What is the diagnosis for a neonate presenting with metabolic acidosis and hyperammonemia resulting from impaired propionyl-CoA to methylmalonyl-CoA conversion?
Propionic acidemia.
What deficiency should be suspected in a patient with elevated methylmalonic acid and neurologic deterioration?
Methylmalonyl-CoA mutase or Vitamin B12 deficiency.
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