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18 Cards in this Set

  • Front
  • Back
Oxidation (remove 2 H by FAD) -- Hydration (to alcohol) -- Oxidation (remove 2 H by NAD) --- Cleavage (2 C)
Step 1 - Removal of 2 hydrogens Oxidation by FAD to a alkene
AcylCoA deH -- take H b.t al-be to put on FADH - make alkene
Step 2 – Hydration of alkene to alcohol
enoylCoA hydratase - add H2O to alkene (make OH)
Step 3 - Removal of 2 hydrogens Oxidation by NAD+ to a ketone
b-H-acylCoA deH -- take H from OH and put on NADH -- make ketone
Step 4 – Cleavage of 2 carbons
Thiolase - add CoA-SH - cut AcetylCoA (2C) for TCA to make NADH-FADH
b-oxidation takes off
b-oxidation takes off
2 carbons at a time
MCAD deficiency (Medium/ Long chain acyl CoA deH def)
Glu to Lys (unstable MCAD) -- dicarboxylic acids in urine
(adding carboxylic acids from w-oxidation FA) --> bind to Ca make kidney stone
beta-oxidation to glycolysis
ATP 30 to 108 need to review steps
unsaturated lipids 1
unsaturated lipids 1
enoylCoA isomerase (5th enz) -- move double bond b.t al-bet from cis to trans
odd-carbon fatty acids
odd-carbon fatty acids
Biotin (B7) help carboxylation (add 1C to even) Coba-lamin (B12) help isomerize to straight chain to Succinyl CoA
C12 coba-lamin deficiency
high methyl-malo-nyl in urine (can't make into straight chain SuccinylCoA --> bind to Ca make kidney stone
unsaturated lipids longer chain
2-4 dienoyl-CoA reductase (6th enz) -- remove H to put on NADPH (PPP) then move double bond (enoylCoA ISOMerase)
Methylmanyl mutate deficiency
bind to Methyl-manyl bind to Ca to make kidney stone
B7 Biotin deficiency
elevation of C3 -- mild symptom -- not a lot of Odd C fat in nature
peroxizomal reaction in high fat diet and long chain FA
flavoprotein dehydrogenase --- electrons are passed from FADH2 (nonsoluble) to molecular oxygen making peroxide WHILE NADH can escape Mitochrondrion and passing e to O2 in mito
Zellweger syndrome
lack of flavoprotein dehydrogenase --- increase in peroxide and potentially hydroxyl radicals.
Ketone Body Formation in the Liver
1.Condensation of two acetyl-CoA’s.
2.Condonsation of third acetyl group to from HMG-CoA
3.Loss of an Acetyl-CoA to form Ketone: Acetoacetate
4.Decarboxylation to Acetone
5.Reduction to beta-hydroxybutarate (main ketone body)
9.Describe in detail the
Ketone Body use in extrahepatic tissue
need to explain in detail Describe in detail the synthesis and utilization of ketone bodies. BUT too tired -- will do in weekend