2. Fatty acids oxidation( beta-oxidation):-
It involves three steps:
a) Activation of fatty acids( Cytosol).
b) Transport of Acyl CoA from cytosol to mitochondria.
c) Beta-oxidation proper( Mitochondrial Matrx).
3. a) Activation of fatty acids:-
site = Cytosol
Acyl Co-A Synthase
Free fatty acids Fatty acyl Co-A
+
Co-A ATP AMP + PPi
Ppi Pyrophosphatase 2 Molecule of inorganic phosphate
H2O
4. b) Transport of fatty acid ( Acyl CoA) From Cytosol
to mitochondria:-
fig:-
5. Carnitine:-
Carnitine is synthesized in liver and kidney.
It require Lysin and metheonine for it’s synthesis.
A large conc. Is present in skeletal muscle.
Diet Synthesized in the body
Source
malonyl CoA inhibitors Carnitine
Functions
Transport of Acyl CoA from cytosol to mitochondria
6. c) Beta-oxidation proper in mitochondrial matrix:
Site = Matrix of mitochondria
a) Oxidation of even chain fatty acid:
Acyl Co-A
Acyl CoA DH FAD
FADH2
Enoyl Co-A
Enoyl CoA hydratase H2O
Beta hydroxy acyl CoA
7. Beta hydroxy acyl CoA
beta OH acyl CoA NAD
NADH2
Beta-keto acyl Co-A
Thiolase CoA
Acetyl CoA
Acyl CoA
Note:-
a) Short Chain fatty acid No need carnitine
b) Medium chain fatty acid
c) Long chain fatty acid. Need carnitine
8. Energetic of palmitic acid condition
Palmitic acid contain 16 carbon molecules.
It undergoes 7 cycles to be completely oxidised and
produced energy.
8 molecules of acetyl CoA produced in whole 7 cycle.
Total energy are given below:
9. Mechanism No. of ATP
produced lost
1) In One Cycle:-
FADH2 produced = 2 ATP
NADH2 produced = 3 ATP
5 ATP
So, In 7 cycles 7 X 5 = 35 ATP’s
2) No. of acetyl CoA produced
in 7 cycles is 8:
: 1 Acetyl CoA = 12 ATP
: 8 Acetyl CoA produced 8X12= 96 ATP
3)During Activation of fatty acid
the energy utilized 2ATP
10. :- Total ATP’s produced = 35+96 =131 ATP’s
and utilized(lost) = 2 ATP’s
:- Net yeild of ATP’s after complete
oxidation of palmitic acid (131 – 2) = 129 ATP’s
11. Disorders:-
1) Carnitine deficiency:
It happens due to immature liver, kidney functions are not normal
and occurs incase of pre-term newborns.
During pregnancy, trauma, burns, requirement of carnitine is
increased and body can’t compensate for that.
During hemodialysis, large amount of carnitine is filtered out.
12. 2) Sudden infants death syndrome :-
Infants die on bed usually during night.
Decreased glucose = utilized with in 2 hrs
beta-oxidation ( normally happen)
Deficiency of medium chain Acyl-CoA dehydrogenase
No Beta-oxidation
No Acetyl CoA
No ATP
Gluconeogenesis Hypoglycemia Death