What distinguishes proteins from carbohydrates and fats?
Presence of nitrogen (~16% of peptide chain)
What bond links amino acids together?
Peptide bond (covalent)
Define dipeptide, tripeptide, polypeptide
2 AA = dipeptide; 3 AA = tripeptide; many AA = polypeptide
Typical length of serum proteins
~100–150 amino acids
Primary protein structure
Amino acid sequence linked by covalent bonds
Secondary protein structure
Winding of polypeptide chain stabilized by hydrogen bonds
Tertiary protein structure
3D folding stabilized by disulfide bonds, H-bonds, electrostatic and hydrophobic interactions
Quaternary protein structure
Association of ≥2 polypeptide chains (e.g., hemoglobin)
Primary site of plasma protein synthesis
Liver (hepatocytes)
Two protein compartments
Intravascular and extravascular
Define nitrogen balance
Balance between protein anabolism and catabolism
Negative nitrogen balance occurs when
Catabolism > anabolism (burns, fever, starvation)
Positive nitrogen balance occurs when
Anabolism > catabolism (growth, pregnancy, recovery)
Enzyme deficiency in PKU
Phenylalanine hydroxylase
Accumulated substance in PKU
Phenylalanine → phenylpyruvic acid
Classic urine odor in PKU
Musty odor
PKU newborn screening test
Guthrie bacterial inhibition assay
MSUD enzyme defect
Keto acid decarboxylase deficiency
Amino acids affected in MSUD
Leucine, isoleucine, valine
MSUD hallmark clinical sign
Maple syrup/burnt sugar odor
Enzyme deficient in homocystinuria
Cystathionine-β-synthase
Elevated amino acids in homocystinuria
Homocysteine and methionine
Major complications of homocystinuria
Thrombosis, osteoporosis, lens dislocation
What reaction detects free amino acids?
Ninhydrin reaction (purple color)