Mnemonic: FAHeem TATtooes because of Histrionic Personality Disorder (HPD) Tyrosine is necessary for biosynthesis of catecholamines, thyroid hormones and melanin pigment. 1. Tyrosinemia type I (Tyrosinosis/Hepatorenal tyrosinemia): Enzyme defect: FAH (Fumaryl-acetoacetate hydroxylase) Last enzyme in the cycle Cabbage like odor Hepatocellular carcinoma Renal tubular acidosis Peripheral… Read More
Vmax is the maximum velocity, or how fast the enzyme can go at full ‘‘speed.’’ Vmax is reached when all of the enzyme is in the enzyme–substrate complex. Km is the substrate concentration at which v = 1/2 Vmax. Km approximately describes the affinity of… Read More
Fatty Acid Activation (Cytoplasm) Before undergoing oxidation, fatty acid must be activated first by conversion into Acyl-CoA by the action of Fatty Acyl-CoA synthase (thiokinase). ATP is converted to AMP (not ADP) – 2 ATP equivalent used Uses Mg2+ as cofactor, Co-ASH and ATP Sites… Read More
Enzymes: Fatty acid synthase (6 enzymes and 1 Acyl carrier protein molecule) Acetyl-CoA carboxylase (rate-limiting enzyme) Starting material: For palmitate synthesis: Acetyl-CoA For odd number carbon long chain fatty acid synthesis: Propionyl-CoA 2 Carbon donor: Malonyl-CoA (donates 2 C and 1 C is thrown out… Read More
Structure Saturated fatty acid: Single bond (“S” for saturated and single bond) Unsaturated fatty acid: Double bond(s), i.e. “D” for Double bonds, Decreased melting temperature and Decreased atherosclerotic risk (by decreasing LDL) Trans-fatty acid: Unsaturated fatty acid which act like saturated fatty acid (hydrogenation of… Read More
