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KIVO Med

Amino-acid metabolism

Follow amino-acid nitrogen and carbon skeletons through catabolism, synthesis, and representative aminoacidopathies.

  • 84 explained questions
  • 33 flashcards
  • 295 estimated course minutes

Updated

What you will learn

  • Relate protein digestion and absorption to amino-acid catabolism.
  • Explain transamination, deamination, ammonia transport, and the urea cycle.
  • Classify amino acids by the fate of their carbon skeleton.
  • Describe biosynthetic families and representative inherited disorders.

Course outline

  1. Orienting amino-acid metabolism

    An overview places nitrogen catabolism, carbon-skeleton fates, and amino-acid synthesis on one map.

    8 min

  2. Protein digestion and absorption

    Gastric and intestinal proteases reduce proteins to absorbable amino acids and small peptides.

    18 min

  3. Removal of the amino group

    Transamination, deamination, and deamidation funnel amino-acid nitrogen toward ammonia.

    24 min

  4. Ammonia transport and disposal

    Glutamine, alanine, urea, and ammonium move and detoxify neurotoxic ammonia.

    20 min

  5. The urea cycle

    Hepatic ureogenesis converts ammonia to urea through five mitochondrial and cytosolic reactions.

    25 min

  6. Urea-cycle regulation and deficiencies

    Substrate supply, hormones, N-acetylglutamate, and enzyme deficiencies modulate ureogenesis.

    34 min

  7. Carbon-skeleton fates

    After nitrogen removal, carbon skeletons converge on seven glucogenic, ketogenic, or mixed intermediates.

    34 min

  8. Decarboxylation and bioactive amines

    PLP-dependent decarboxylases release CO₂ and form amines with distinct physiological roles.

    12 min

  9. Synthesis and biosynthetic families

    Central-metabolism precursors organise amino acids into six families, after which specific reactions build each member.

    42 min

  10. Glutamine: nitrogen shuttle and cellular fuel

    Glutamine connects peripheral production, blood transport, nitrogen disposal, and carbon use across several organs.

    36 min

  11. Aminoacidopathies: reasoning from the metabolic block to the patient

    Understand separately the enzyme deficiencies and transport defects that cause amino acids or their derivatives to accumulate.

    42 min

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