Vitamin Deficiencies and Impairments in Amino Acid Metabolism

 

Vitamin Impaired Reaction Enzyme Affected Reaction Catalyzed Pathway/Consequence
Vitamin B6 (PLP) Transamination of amino acids Aminotransferases (e.g., ALT, AST) Alanine + α-Ketoglutarate ↔ Pyruvate + Glutamate Impaired amino acid catabolism and gluconeogenesis
Conversion of glutamate to γ-aminobutyric acid (GABA) Glutamate decarboxylase Glutamate → GABA + CO₂ Impaired neurotransmitter synthesis
Conversion of homocysteine to cystathionine and cysteine Cystathionine β-synthase Homocysteine + Serine → Cystathionine Homocysteine accumulation
Thiamine (B1) Oxidative decarboxylation of branched-chain α-keto acids Branched-chain α-keto acid dehydrogenase Valine, Leucine, Isoleucine α-keto acids + NAD⁺ → Acyl-CoA + NADH + CO₂ Impaired branched-chain amino acid catabolism
Folate (B9) Conversion of homocysteine to methionine (methyl group transfer) Methionine synthase (with Vitamin B12) Homocysteine + 5-Methyl-THF → Methionine + THF Homocysteine accumulation, disrupted methylation
Vitamin B12 Conversion of methylmalonyl-CoA to succinyl-CoA Methylmalonyl-CoA mutase Methylmalonyl-CoA → Succinyl-CoA Impaired odd-chain fatty acid and amino acid metabolism
Conversion of homocysteine to methionine Methionine synthase Homocysteine + 5-Methyl-THF → Methionine + THF Homocysteine accumulation, impaired SAM synthesis
Riboflavin (B2) Oxidation of L-amino acids (e.g., proline, lysine) L-amino acid oxidase L-Amino acid + FAD → α-Imino acid + FADH₂ Impaired amino acid degradation
Biotin Carboxylation in amino acid metabolism Propionyl-CoA carboxylase Propionyl-CoA + CO₂ + ATP → Methylmalonyl-CoA + ADP + Pi Impaired catabolism of valine, isoleucine, threonine

 

Key Reactions Explained:

  1. Vitamin B6 (PLP):
    • Aminotransferases (ALT, AST): Vital for interconversion of amino acids and keto acids in transamination reactions.
    • Cystathionine β-synthase: Converts homocysteine and serine to cystathionine, and then to cysteine, preventing homocysteine buildup.
    • Glutamate decarboxylase: Critical for GABA synthesis, affecting neurotransmission.
  2. Thiamine (B1):
    • Branched-chain α-keto acid dehydrogenase: Catalyzes oxidative decarboxylation in branched-chain amino acid catabolism (e.g., valine, leucine).
  3. Folate (B9) and Vitamin B12:
    • Methionine synthase: Facilitates methylation cycles by regenerating methionine from homocysteine.
  4. Biotin:
    • Propionyl-CoA carboxylase: Handles catabolism of amino acids generating propionyl-CoA (e.g., valine, isoleucine).

 

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