Beta Oxidation Of Fatty Acids Flashcards
Beta oxidation location
Inner mitochondria or matrix
Principals means by which FA oxidized to forms ATPs
Beta oxidation
Process of beta oxidation
Removal of 2 carbon atoms at a time
Removals start at COOH (carboxyl end)
Major end product of beta oxidation
Acetyl CoA
Very first reaction of beta oxidation takes place in the
Cytoplasm
The chemical reactions in beta oxidation are usually opposite to
Lipogenisis
Addition of 2 carbons at a time
Lipogenesis
Lipogenesis location
Cytoplasm
Lipogenesis
Substrate
Acetyl CoA
All even number fatty acids the end products
Acetylcoa
If odd number fatty acids
It will undergo usual beta oxidation but when you already have 5 remaining carbon atoms minus 2 3 carbon atoms, profunyl CoA, then methyl malonyl coa, then succinyl coa
3 carbon atom molecule
Profunyl coA
Intermediate of krebs cycle
Succinyl coa
Reaction 1
Free fatty acid +CoA + ATP to
Fatty Acyl CoA
Reaction 1 location
Cytoplasm
Reaction 1 enzyme
Thiokinase (Acyl CoA synthetase)
ATP reaction
ATP to AMP + PPI + Heat
Enzyme
Pyrophosphate
ATP reaction
PPi to Pi + Pi + heat
Enzyme
Pyrophosphatase
Reaction 2
Carnitine transport system
Reaction 2.1
Fatty Acyl CoA + Carnitine to
Acylcarnitine (Fatty Acyl Carnitine Complex)
Reaction 2.1
Enzyme
Carnitine Palmitoyl Transferase 1
Rate limiting enzyme
Location of the enzyme in reaction 2.1
Outer mitochondrial membrane
Enzyme in reaction 2.1 inhibited by
Malonyl CoA
Intermediate in extramitochondrial lipogenesis
Malonyl CoA
Kaya hindi pwedeng magkasbay ang lipogenesis at beta oxydation
Reaction 2.1
Location of the reaction:
Intermitochondrial space
Rate limiting step and commited step
Reaction 2.1
Reaction 2.2
Acylcarnitine goes
Inside the super inside of the mitochondria (mitochondrial matrix)
Reaction 2.2
Enzyme
Carnitine Acylcarnitine Translocase
Reaction 2.2
Location of the enzyme
Inner mitochondrial membrane
Reaction 2.2
Type of enzyme
Co transporter
Anti port
2 molecule of acylcarnitine transported to the mitochondrial matrix
Co transporter
1 molecule of carnitine transported to the mitochondrial intermembrane space
Anti port
Reaction 2.2
Location of reaction
Intermitochondrial space to mitochondrial matrix
Reaction 2.3
Acylcarnitine to
Acyl CoA + Carnitine
Reaction 2.3
Acyl CoA goes to
Reaction 3.1
Reaction 2.3
Carnitine transported to
Back to intermitochondrial space
Reaction 2.3
Enzyme
Carnitine palmitoyl transferase 2
Reaction 2.3
Location of enzyme
Inner mitochondrial matrix
Reaction 2.3
Location of reaction
Mitochondrial matrix
Reaction 2.4
Carnitine goes back to the intermitochondrial space
Reaction 2.4
Enzyme
Carnitine Acylcarnitine translocase
Carnitine synthesized from
Lysine
Methionine
Sufficient doses of carnitine
Enough substance to proceed to beta oxidation
More carnitine
More FA oxidized
Less FA converted to fats
Reaction 3
Oxidative step
Reaction 3
Location
Mitochondrial matrix
Reaction 3.1
1st oxidative step
Reaction 3.1 1st oxidative step
Fatty acyl CoA to
Unsaturated Acyl CoA
Reaction 3.1
Enzyme
Acyl CoA dehydrogenase
Reaction 3.1
Coenzyme
FAD
Reaction 3.2
Hydration step
Reaction 3.2hydration step
Unsaturated acyl CoA to
Beta Ketoacyl CoA
Reaction 3.2 hydration step
Enzyme
Enoyl CoA Hydratase
Reaction 3.2 hydration step
Reaction process
Water added
Reaction 3.3
2nd oxidative step
Reaction 3.3 2nd oxidative step
Beta Hydroxyacyl CoA to
Beta Ketoacyl CoA
Reaction 3.3 2nd oxidative step
Enzyme
Beta hydroxyl CoA dehydrogenase
Reaction 3.3 2nd oxidative step
Coenzyme
NAD
In reaction 3 how many ATP produced per cycle?
5ATP
2 from FADH
3 from NADH
Reaction 4
Thiolysis
Reaction 4 Thiolysis
Beta Ketoacyl CoA to
Fatty Acyl CoA + Acetyl CoA
Reaction 4 Thiolysis
Fatty acyl CoA goes to
Reaction 3 hanggang maubos ito
Reaction 4 Thiolysis
Acetyl CoA goes to
Lipogenesis
Reaction 4 Thiolysis
Enzyme
Beta Keto Thiolase
Other types of fatty acid oxidation
Peroximal oxidation
Alpha oxidation
Omega oxidation
Oxidation of VERY LONG CHAIN FATTY ACIDS (C20-C22)
Modified form of Beta Oxidation
Peroximal Oxidation
Peroximal Oxidation
End products
Acetyl CoA
H2O2 (peroxide)
Removes ONE carbon atom at a time starting from COOH end
Does not generate high energy phosphate
CoA not required
Alpha Oxidation
Minor pathway
Cytochorme P450 involved
Omega oxidation
Omega oxidation
Location
Endoplasmic Reticulum
Episodic periods of hypoglycemia
Decreased gluconeogenesis
Carnitine deficiency
Hypoglycemia
Affected organ is liver
Inherited
Deficiency of Carnitine Palmitoyl CoA transferase 1
Affects the skeletal muscles
Necrosis with myoglobinuria
Deficiency of carnitine palmitoyl CoA transferase 2
Defectin alpha oxidation
Accumulation of Phytanic Acid
Refsum’s disease
Caused by eating unripe fruit (akee tree)
Fruit with hypoglycin
Jamaican vomiting sickness
Inactivates. Medium and short chain Acyl CoAdehydrogenase
Beta oxidation inhibited
Hypoglycin
Absence of peroxisomes in the tissue
Accumulation of Polyenoic Acids (C26-C38) in the brain tissue
Impaired bile acid and other lipid synthesis
Zellweger’s cerebrohepatorenal syndrome