Beta-oxidation is the metabolic process where fatty acids are broken down in the mitochondria to produce ATP, crucial during fasting or prolonged exercise. The pathway includes four main enzymatic steps: oxidation, hydration, a second oxidation, and cleavage, ultimately yielding significant energy. Understanding beta-oxidation is important for diagnosing metabolic disorders like carnitine deficiency and medium-chain acyl-CoA dehydrogenase deficiency.

1. Beta Oxidation Of Fatty Acid
Name: Momin Siddique
Reg.No: SU-23-01-081-106
Date: 30 july 2024

2. Introduction to Beta Oxidation
• Definition: Beta-oxidation is the metabolic
process by which fatty acids are broken down
in the mitochondria to produce energy.
• Importance: Provides a significant source of
ATP, especially during fasting or prolonged
exercise.

3. Overview of Fatty Acid Metabolism
• Lipolysis: Breakdown of triglycerides into free
fatty acids and glycerol.
• Transport into Mitochondria: Fatty acids are
transported into the mitochondria via the
carnitine shuttle.

4. The Beta Oxidation Pathway
• Location: Mitochondrial matrix.
• Steps Overview:
Oxidation
Hydration
Oxidation (second)
Thiolysis

5. Step:1 Oxidation
• Enzyme: Acyl-CoA dehydrogenase.
• Reaction: Conversion of acyl-CoA to trans-
enoyl-CoA with the reduction of FAD to
FADH2.
• Purpose: Formation of a double bond
between the alpha and beta carbons.

6. Step:2 Hydration
• Enzyme: Enoyl-CoA hydratase.
• Reaction: Addition of water across the double
bond of trans-enoyl-CoA to form L-3-
hydroxyacyl-CoA.
• Purpose: Formation of a hydroxyl group.

7. Step:3 Oxidation(again)
• Enzyme: 3-Hydroxyacyl-CoA dehydrogenase.
• Reaction: Conversion of L-3-hydroxyacyl-CoA
to 3-ketoacyl-CoA with the reduction of NAD+
to NADH.
• Purpose: Formation of a keto group.

8. Step:4 Cleavege
• Enzyme: Beta-ketothiolase.
• Reaction: Cleavage of 3-ketoacyl-CoA into
acetyl-CoA and a shortened acyl-CoA.
• Purpose: Production of acetyl-CoA for the
citric acid cycle and a shorter acyl-CoA for
further oxidation.

9. Pathway of Beta Oxidation of Fatty Acid

10. Energy Yield
• ATP Production: Each round of beta-oxidation
generates:
1 FADH2 (1.5 ATP)
1 NADH (2.5 ATP)
1 Acetyl-CoA (produces 10 ATP in the citric
acid cycle)
• Overall: Approximately 106 ATP per palmetic
acid molecule (16 carbons).

11. Regulation Of Beta Oxidation
• Enzymes Regulated: Acyl-CoA dehydrogenase,
carnitine acyltransferase.
• Factors Affecting Regulation:
Availability of fatty acids.
Hormonal signals (e.g., insulin, glucagon).

12. Clinical Revelance
• Disorders:
Carnitine deficiency: Impaired fatty acid transport
into mitochondria.
Beta-oxidation disorders: e.g., Medium-chain
acyl-CoA dehydrogenase deficiency (MCADD).
• Impact: Energy production issues,
hypoglycemia, muscle weakness.

13. Conclusion
• Summary: Beta-oxidation is crucial for energy
production from fatty acids, involving a series
of enzymatic steps in the mitochondria.
• Implications: Understanding beta-oxidation
helps in diagnosing and managing metabolic
disorders.

14. Refrences
• Chatgpt
• Essentials of Medical Biochemistry by
Mushtaq Ahmed
• Google
• Sciencedirect.com