Mitochondrial fatty acid transport pathway

The **mitochondrial fatty acid transport pathway** refers to the series of molecular events that enable long-chain fatty acids to enter mitochondria for β‑oxidation. This process is essential because long-chain acyl-CoA molecules cannot cross the inner mitochondrial membrane directly. Instead, they are converted into acylcarnitines by **carnitine palmitoyltransferase 1 (CPT1)** on the outer mitochondrial membrane. Acylcarnitines are then shuttled across the inner membrane by **carnitine-acylcarnitine translocase (CAC/SLC25A20)** and reconverted into acyl-CoAs by **CPT2** on the matrix side. The resulting acyl-CoAs undergo β‑oxidation within mitochondria to generate acetyl-CoA for energy production via the TCA cycle and oxidative phosphorylation[5][6]. Defects in any component can lead to significant metabolic diseases characterized by impaired energy homeostasis. Note: The term "Mitochondrial fatty acid transport pathway" describes a multi-protein process rather than a single molecule/receptor; it is not itself a canonical drug target but includes several well-defined protein targets such as CPT1A/B/C, SLC25A20 (CAC), and CPT2[5]. Thus, **is_incorrect = true**, since it does not refer specifically enough to an individual molecular entity suitable for structured drug-target information without further specification.

Other names

Mitochondrial fatty acid importCarnitine shuttle system (refers to the main mechanism within this pathway)Fatty acid mitochondrial uptake pathway

For drugs targeting components of this pathway, mechanisms include inhibition or activation of carnitine palmitoyltransferases, modulation of acylcarnitine levels, or supplementation with L-carnitine to bypass defects[5].