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2. . Detoxification of monoamines catalysed by mitochondrial outer membrane MAO. . R-CHNH2. . . . O2. H2O2. R-CH=NH. . . NH3. R-CHO. . R-COOH. . b-oxidation. CO2. . ATP, HCO3-. UREA. urea cycle. . MONOAMINE OXIDASEFlavin-containing amine oxidase. (imine). . inner membrane. . outer membrane.
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3. Alcohol induced steatohepatitis 3
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8. 8 8. Hemoprotein toxicity diseases caused by heme or bilirubin A) Rhabdomyolysis releases myoglobin and heme and causes kidney mitochondrial damage
B) Kernicterus:- bilirubin causes neonatal brain mitochondrial damage
9. 9 A) Myoglobin mediated rhabdomyolysis rarely caused by statins
10. 10 ie. Kernicterus (brain damage caused by bilirubin in the newborn (hyperbilirubinemia))
11. 11 Metabolic pathway of heme degradation to bilirubin and detoxification of bilirubin by glucuronidation.
12. 12 9. HEME BIOSYNTHESIS BYMITOCHONDRIA AND GENETIC DISEASES
13. 13 A) HEME BIOSYNTHESISand porphyria diseases
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15. Heme synthesis Heme required for synthesis of mitochondrial cytochromes and endoplasmic reticular P450s.
Heme required for bone marrow synthesis of hemoglobin and muscle myoglobin
STEP 1 for heme synthesis is the synthesis of aminolevulinic acid (ALA) from succinyl CoA of the citric acid cycle and glycine
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20. 20 B) PORPHYRIAS
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28. 28 C) OXIDATIVE DEGRADATION OF HEME TO BILIRUBIN
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33. 33 a) N-Catabolism of Amino Acids (The Urea Cycle) and the Detoxification of Ammonia (very toxic)
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36. 36 IV: Peroxisomes Function ß-oxidation of fatty acids but oxidase forms H2O2
Glyceraldehyde is metabolised to D-glycerate and glucose via glycolate (glycolate oxidase forms H2O2 and glyoxylate)
Catalase detoxifies H2O2 to form oxygen and H2O and cooxidises other substrates, including phenols, formic acid, formaldehyde, and methanol.
Glyoxylate detoxified by peroxisomal alanine:glyoxylate transaminase to form glycine & pyruvate. Otherwise glyoxylate would be oxidised by lactate dehydrogenase to oxalic acid which causes oxalate stone formation & kidney damage.
Biosynthesis of bile acids and ether phospholipids
37. 37 V: CYTOSOLIC FRACTION N-catabolism of purines
Glycogen synthesis and glycogenolysis
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