Final answer:
Endogenous synthesis of dietary carnitine plays a crucial role in the transport of fatty acids in Primary Carnitine Deficiency. The process involves the formation of a fatty acyl-carnitine complex and its transfer through the inner mitochondrial membrane. In the mitochondrial matrix, the complex is hydrolyzed to yield fatty acyl-CoA and carnitine.
Step-by-step explanation:
The endogenous synthesis of dietary carnitine helps in the transport of fatty acids in Primary Carnitine Deficiency. Carnitine reacts with activated fatty acids in the presence of carnitine acyl transferase-I, forming a fatty acyl-carnitine complex and CoA-SH. This complex is then transported through the inner mitochondrial membrane via carnitine acyl carnitine translocase. In the mitochondrial matrix, another enzyme called carnitine acyl transferase-II hydrolyzes the fatty acyl carnitine to give fatty acyl-CoA and carnitine.