Human Acyl-CoA Dehydrogenase, Long Chain (ACADL) Protein

Background

ACADL , also known as long-chain acyl-CoA dehydrogenase (LCAD), is a mitochondrial enzyme critical for the β-oxidation of long-chain fatty acids. It catalyzes the first step in the oxidative breakdown of long-chain fatty acyl-CoAs, facilitating their conversion into energy through the tricarboxylic acid (TCA) cycle and electron transport chain. ACADL is predominantly expressed in energy-demanding tissues such as the liver, heart, and skeletal muscle, where fatty acid metabolism is essential for maintaining energy homeostasis during fasting and sustained physical activity. Dysregulation or mutations in the ACADL gene can result in long-chain acyl-CoA dehydrogenase deficiency, a rare metabolic disorder characterized by hypoglycemia, muscle weakness, and lipid accumulation due to impaired fatty acid oxidation. ACADL also plays a role in lipid signaling and mitochondrial health, contributing to cellular responses to metabolic stress. Its enzymatic activity underscores its importance in energy metabolism, lipid regulation, and mitochondrial function, making it a key target for studies on metabolic diseases and energy balance.