Essential Amino Acids and Proteins

Essential Amino Acids and Proteins

Essential Amino Acids and Proteins

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Essential Amino Acids - The VIP Building Blocks

  • Indispensable dietary AAs; body cannot synthesize them de novo sufficiently for growth/maintenance.
  • 📌 Mnemonic: PVT TIM HALL
    • Phenylalanine, Valine, Threonine
    • Tryptophan, Isoleucine, Methionine
    • Histidine (essential, esp. infants, uremia)
    • Arginine (conditionally essential: trauma, sepsis)
    • Leucine, Lysine
  • Metabolic Fates:
    • Purely Ketogenic: Leucine, Lysine.
    • Both Glucogenic & Ketogenic: Phenylalanine, Isoleucine, Tryptophan, Threonine.
  • Vital for protein synthesis, N-balance, biomolecule precursors.
  • Essential Amino Acid Mnemonic PVT TIM HALL

Maple Syrup Urine Disease (MSUD): Autosomal recessive; deficient branched-chain α-ketoacid dehydrogenase. Affects Leucine, Isoleucine, Valine (LIV).

Protein Structure - From Chains to Shapes

  • Primary (1°): Amino acid sequence; peptide bonds. Determines final protein shape.
  • Secondary (2°): Local folding (α-helix, β-sheet) via backbone H-bonds.
    • α-helix: Right-handed coil. H-bonds between C=O ($n$) & N-H ($n+4$). 📌 Proline: helix breaker.
    • β-sheet: Extended strands, H-bonded (parallel/antiparallel).
  • Tertiary (3°): Overall 3D shape of one polypeptide. Interactions: disulfide, hydrophobic, ionic, H-bonds.
  • Quaternary (4°): Arrangement of multiple subunits (e.g., Hemoglobin). Protein Structure Levels

⭐ Denaturation (heat, pH, chemicals) disrupts 2°, 3°, 4° structures, sparing 1° (peptide bonds).

Protein Functions & Metabolism - The Body's Workhorses

Protein digestion and absorption in the GI tract

  • Functions:
    • Structural: Collagen (connective tissue), keratin (hair/nails)
    • Enzymatic: Catalysts (pepsin, DNA polymerase)
    • Transport: Hemoglobin (O₂), albumin (fatty acids, drugs), transferrin (Fe³⁺)
    • Immune Defense: Antibodies (immunoglobulins)
    • Hormonal Regulation: Insulin, glucagon, GH
    • Movement: Contractile (actin, myosin)
    • Acid-Base Balance: Buffers (hemoglobin)
    • Receptors/Channels: Membrane proteins
  • Metabolism:
    • Digestion:
      • Stomach: Pepsinogen → Pepsin (HCl)
      • Small Intestine: Pancreatic (trypsin) & brush border enzymes
    • Absorption: Active transport of AAs, di/tripeptides
    • Nitrogen Balance: Reflects body protein status.
      • Positive (N intake > N excretion): Growth, pregnancy.
      • Negative (N intake < N excretion): Starvation, trauma.
    • Amino Acid Pool: ~100g free AAs; sources (diet, breakdown), fates (synthesis, energy).
    • Urea Cycle (Liver): Converts toxic $NH_3$ from AA catabolism to urea for renal excretion.

      ⭐ Ornithine Transcarbamylase (OTC) deficiency: most common urea cycle disorder (X-linked). Hyperammonemia, encephalopathy.

Nutritional Aspects & Disorders - Fueling & Failing

  • Protein Quality Metrics:
    • Nitrogen Balance: $N_{in} - (UUN + \textbf{4g})$. Positive (anabolism), Negative (catabolism).
    • Biological Value (BV): (N retained / N absorbed) × 100. Egg protein BV ≈ \textbf{100}.
    • Net Protein Utilization (NPU): BV × Digestibility Coefficient.
    • Protein Efficiency Ratio (PER): Weight gain (g) / Protein intake (g).
  • Protein-Energy Malnutrition (PEM):
    • Kwashiorkor ("sickness of weaning child"): ↓ Protein > ↓ Energy. Edema, hypoalbuminemia, fatty liver, dermatosis (flaky paint), hair (flag sign).
    • Marasmus ("dying away"): ↓ Energy > ↓ Protein. Severe wasting, "old man" face, alert & irritable. No edema.
    • Markers: ↓ Serum albumin, ↓ prealbumin (transthyretin - T½ \textbf{2-3 days}).
  • Selected Aminoacidopathies (Metabolic Failure):
    • PKU: ↓ Phenylalanine hydroxylase. Mousy odor. Guthrie test.
    • MSUD: ↓ Branched-chain α-ketoacid dehydrogenase. Burnt sugar urine odor.
    • Alkaptonuria: ↓ Homogentisate oxidase. Dark urine, ochronosis.
    • Homocystinuria: ↓ Cystathionine β-synthase. Lens (↓ & in), Marfanoid, thrombosis.

⭐ > In Kwashiorkor, edema is primarily due to hypoalbuminemia leading to decreased plasma oncotic pressure.

Kwashiorkor and Marasmus Comparison

High‑Yield Points - ⚡ Biggest Takeaways

  • Essential amino acids (PVT TIM HALL) must be obtained from diet; Arginine & Histidine are semi-essential.
  • Kwashiorkor (protein deficiency, edema) vs. Marasmus (calorie & protein deficiency, wasting).
  • Phenylketonuria (PKU): phenylalanine hydroxylase deficiency; musty odor, CNS symptoms.
  • Maple Syrup Urine Disease (MSUD): deficiency of branched-chain α-ketoacid dehydrogenase.
  • Alkaptonuria: homogentisate oxidase defect; ochronosis, black urine on standing.
  • Biological Value (BV) & NPU measure protein quality; positive nitrogen balance indicates anabolism_._

Practice Questions: Essential Amino Acids and Proteins

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Protein metabolism after trauma is characterized by the following except:

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Flashcards: Essential Amino Acids and Proteins

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_____ have the highest NPU value and that is why it is considered as a reference protein

TAP TO REVEAL ANSWER

_____ have the highest NPU value and that is why it is considered as a reference protein

Eggs

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