NH, FAD 0—CH2 ADP 0=P-0 Struktural elements 0 0=P-0 OH OH of the coenzymes: 0 FAD und FMN CH2 HC-OH Ribitol FMN HC-OH Ribo- flavin HC-OH 个 Transfer of H2 HaC. .0 Isoallo- (H+H) NH xacine HgC
Struktural elements of the coenzymes: FAD und FMN ADP Isoalloxacine Ribitol Transfer of H2 (H- + H+ )
R-CH2-CH2-CH2-C-S-CoA Acyl-CoA β-Oxidation Acyl-CoA-Dehydrogenase FAD FADH2 R-Cz-C-S-coA --总--sm 0 Acyl-CoA △2.trans--Enoyl--CoA CH-c-s-COA/ HOH Enoyl-CoA- Hydratase Acetyl-CoA 3-Ketothiolase CoA-SH HO 3-Ketoacyl-CoA L-3-Hydroxyacyl-CoA NADH+H+ NAD+ L-3-Hydroxyacyl-CoA-Dehydrogenase
b - Oxidation
H2C-CHa S-Co Propionyl-CoA Degradation of ATP H20 Propiony-CoA (Biotin) ADP P uneven numbered Carboxylase fatty acids HC-CHg -S-CoA D-Methylmalonyl-CoA Methylmalonyl- CoA Racemasease L-Methyimalonyl-Co/ L-Methylmalonyl- Vitamin B2 CoA Mutase (Cobalamin) Citric acid cycle← Succinyl-CoA
Degradation of uneven numbered fatty acids Vitamin B12 (Cobalamin) Propiony-CoA Carboxylase MethylmalonylCoA Racemasease L-MethylmalonylCoA Mutase Citric acid cycle
Lindlsture 心 (Ochdeca SCoA di2 asdiure） 3 NAD++3 FAD +3 CoA-SH rounds of B oxi 3 NADH+3 FADH2 +3 Acetyl-CoA Problem 1: B.y double bond Degradation of SCo/ unsaturated noyl-CoA SCoA fatty acis NAD*+FAD+CoASH one round of B oxidation the first oxidation of NADH FADH2+Acetyl-CoA the next round Problem 2: △4 double bond NADPH +H* NADP* 2.4-dienoyl- (E.coli) 2-enoyl-CoA Continuation of B oxidation
Degradation of unsaturated fatty acis
Degradation of very long chain fatty acids Very long chain fatty acids will be shortened in the peroxisome before regular B oxidation occurs in the mitochondrion.This peroxisomal function is essential,as misfunction of the peroxisome leads to Adrenoleukodystrophy,a genetically transmitted disease.This disease leads to dementia during childhood and finally death.Since the gene is x chromosomally located,usually only boys or young man,respectively,are affected
Degradation of very long chain fatty acids Very long chain fatty acids will be shortened in the peroxisome before regular b oxidation occurs in the mitochondrion. This peroxisomal function is essential, as misfunction of the peroxisome leads to Adrenoleukodystrophy, a genetically transmitted disease. This disease leads to dementia during childhood and finally death. Since the gene is x chromosomally located, usually only boys or young man, respectively, are affected