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β- Oxidation of fatty acids ط      Transport of fatty acids into mitochondria

β- Oxidation of fatty acids ط      Transport of fatty acids into mitochondria ط       β- Oxidation of fatty acids ط       steps and energetic                                      D4 381-7 . Introduction 1.        FAs consist of 1 CH3 methyl end, Carbon chain, COOH carboxyl end

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β- Oxidation of fatty acids ط      Transport of fatty acids into mitochondria

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  1. β-Oxidation of fatty acids ط     Transport of fatty acids into mitochondria ط      β- Oxidation of fatty acids ط      steps and energetic                                      D4 381-7 

  2. Introduction 1.        FAs consist of 1 CH3 methyl end, Carbon chain, COOH carboxyl end 2.        Short chain SC-FAs (>8C), medium chain MC-FAs (8-12C), Long Chain LC-FAs (<12C) 3.        Saturated FAs (straight chain), Unsaturated FAs (has == double bond; cis same side H & trans opposite side H) 4.        Odd chain FAs consist of odd number of carbon chain

  3. β-FAO (for saturated LC- & MC-FA, 1st cut between C2 & C3) fig9.19 End Retic / cytosol: FA AACoA S-ase (–2ATP/CoA)  FACoA outer mitoch memb: FACoA + Carnitine CPT1 (+CoA)  FACarnitine inner mitoch memb: FACarnitine  T-lase FA + Carnitine inner mitoch memb: FA CPT2 (–CoA)  FACoA mitoch matrix: FACoA ACoADH (+FADH2)  trans-2ECoA ECoAH-tase (–H2O)  L-HACoA L-HACoA HACoADH (+NADH) β-KACoA β-KACoA β-KACoA T-alse (+CoA)  FACoA (–2C) + ACoA (to TCA cycle) * one turn produce 5 ATP (2 from FADH2 & 3 from NADH) * one ACoA enters TCA cycle produce 12 ATP * complete oxid of 16C FA chain (Palmitoyl Acid) produce 129 ATP (2 ATP used for entery into mitoch) * Reactions regulated by ACoA, cofactors, Carnitine

  4. Oxidation of fatty acids and clinical aspects ط      Saturated and unsaturated fatty acid oxidation ط     Oxidation of odd number fatty acids ط     α- oxidation and ω-oxidation of fatty acids ط     Clinical correlation :                             Refsum’s disease                             Genetic deficiencies in Carnitine transport                                      D4 381-7 

  5. Unsaturated FAO (for LC-FA, to overcome == bouble bonds) fig9.22 LCoA => β-Oxid (+3CoA) => cis·3ECoA ECoA I-asetrans·2ECoA => β-Oxid (+CoA) => cis·4ECoA cis·4ECoA ACoADHtrans·2-cis·4ECoA DECoA R-ase (–NADPH) trans·3ECoA trans·3ECoA ECoA I-asetrans·2ECoA => β-oxid (+5ACoA) α-FAO (1st cut between C1 & C2) Hydroxylation of saturated SC-FA (Mitoch & End Retic) Hydroxylation of LC-FA only for Sphingolipids ω-FAO (at methyl end) Hydroxylation of saturated MC-FA (in End Retic) and for Hydroxy FA (phospholipids) Odd-Chain FAO (modification of α-oxid) same as from AA => α-oxid => PCoA => carboxylation => MMCoA => conversion => SCoA

  6. Clinical Correlation of FAOcc.9.4 Genetic deficiency in Carnitine or CPT cause mild-recurrent muscle cramps to severe weakness & death ·         Carnitine Primary Deficiency: o        No LC-FAO (heart / muscle) o        Diagnosed by low plasma carnitine o        Treated by Dietary Carnitine ·         Carnitine Secondary Deficiency: o        Accumulation of FAcOA / FACarnitine plasma lead to excretion in urine o        Drainage of body from carnitine lead to impaired tissue uptake of carnitine o        Cannot treat with Dietary carnintine ·         CPT genetic deficiency: o        Most common is deficiency of CPT2 o        Lead to muscle weakness during prolonged exercise o        Fatal for children (hypoketotic, hypoglycaemia, hyperammonemia) o        Treated by avoiding starvation and use low LC-FA in diet ± dietary MC-TG (carnitine independent)

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