Final answer:
Carnitine is essential for the transport of fatty acids into mitochondria for energy production. Loss of carnitine leads to decreased fatty acid oxidation, manifesting as muscle weakness, heart problems, and liver dysfunction.
Step-by-step explanation:
Function and Loss of Carnitine
Carnitine, a compound critical for the transport of fatty acids into the mitochondrial matrix for oxidation, plays an essential role in energy production. The carnitine mechanism involves the formation of a fatty acyl carnitine complex in the presence of the enzyme carnitine acyl transferase-I. This complex is then shuttled across the inner mitochondrial membrane through a transport protein called carnitine acyl carnitine translocase. On the membrane's inner surface, carnitine acyl transferase-II hydrolyzes the complex to release fatty acyl-CoA for further oxidation and free carnitine for reuse.
Loss of carnitine can lead to an inability to transport fatty acids into mitochondria, causing a reduction in energy production since fatty acids are a significant source of fuel for cells. Therefore, a deficiency in carnitine can manifest as muscle weakness, heart problems, and liver dysfunction due to the accumulation of unoxidized fatty acids in the cytoplasm.