Question

Why are the symptoms of carnitine palmitoyl transferase|| deficiency more severe during fasting?

Short answer

ATP is generated through fatty acids during starvation, which is why carnitine palmitoyl transferase II deficiency symptoms are more severe during fasting.

Step-by-step solution

Explanation of Concept

Carnitine palmitoyl transferase II is a peripheral inner mitochondrial membrane protein that is found in all tissues that oxidize fatty acids as a monomeric protein.

Process of Converting Fats into Energy 

The carnitine palmitoyl transferase || enzyme is required for fatty acid oxidation, which is the process by which fats are metabolized and converted into energy. It transfers the fatty acids from cytosol to mitochondria.

Defects in tissues 

A carnitine palmitoyl transferase defect prevents tissues, such as muscle, from producing ATP when needed. Tissues can't get enough fuel to function properly and die off faster than usual.

Hence, Tissues can't get enough fuel to function properly and die off faster than usual.

During Starvation 

The symptoms of the carnitine palmitoyl transferase II enzyme are harsher during famine than under normal circumstances because there is a greater demand for ATP, and fatty acids are used to generate ATP during starvation.