Glycerol > Glycerol-3P via?
TG > 3 F.A. + glycerol via ?
- Glycerokinase
- TG lipase
How are F.A. transported in circulation
FA-albumin complex and FABP
Does FA oxidation occur in RBC/Brain
No
B-oxidation location and mode
matrix, aerobic…but activation occurs in cytoplasm
Activation:
Transport:
Oxidation:
1- via acyl CoA synthetase(2 ATP)
2- Outer IMM…CAT-1(Acyl CoA > Acyl-carnitine)
Inner IMM…CAT-2(Acyl carnitine > Acyl CoA)
3- Ox, hydration, ox, thiolysis
T/F: Oxidation of unsaturated F.A needs more energy than saturated F.A.
False
2 additional enzymes needed in oxidation of unsaturated F.A.
Isomerase(Cis > Trans)
Enoyl reductase(= position)
T/F: Odd chain F.A oxidation occurs in plants
True
T/F: All forms of F.A. oxidation are energy producing
False. Peroxisomal oxidation is not
5 differences between P-ox and B-ox
1- In peroxisome, not matrix
2- Independent of carnitine
3- 1st oxidation via FAD-linked acyl oxidase
4- Bifunctional enzyme for hydration and 2nd ox
5- Final product: acetyl CoA + octanoic acid
O-oxidation produces _
Examples:
- dicarboxylates
- Suberic acid(C8) from octanoic acid. Catabolism of prostaglandins, cholesterol, xenobiotics
@-oxidation involves _
Disease? Due to?
Effects?
- Phytanic acid
- Refsum’s disease, defect in hydroxylase/dioxygenase
- Tremor, poor night vision
- Why can’t phytanic acid undergo B-ox?
- What happens?
- It is a tertiary alc at the L-hydroxy intermediate.
- Phytanic acid > Pristanic acid via hydroxylase/dioxygenase then proceeds with B-ox
F.A. pool mainly taken up by
liver
Ketogenesis occurs in _
When _
- Matrix, only in liver
- High F.A. in liver
T/F: All tissues can use KB
False. RBC’s(no mitochondria) and liver(no transferase) cant, brain can
T/F: KB provide direct source of energy over F.A.
True
Acetoacetate activated via?
Acetoacetyl CoA > 2 acetyl CoA via?
- Acetoacetate-succinyl CoA transferase.
- Thiolase
How is CAT-2 regulated
It isn’t
MCAD defect leads to
SIDS
