Carnitine palmitoyltransferase 1B (CPT1b)

CPT1b is a mitochondrial enzyme that catalyzes the transfer of long-chain fatty acyl groups from coenzyme A to L-carnitine, forming acylcarnitines. This reaction is essential for the transport of long-chain fatty acids from the cytoplasm into mitochondria for β-oxidation and energy production. CPT1b is considered a rate-limiting step in mitochondrial fatty acid oxidation (FAO), especially in tissues with high energy demands such as heart and skeletal muscle. Deficiency or inhibition of CPT1 can impair mitochondrial FAO, leading to hypoketotic hypoglycemia during fasting or illness due to inability to utilize fats for energy. It has also been explored as a target for metabolic therapies aimed at treating cardiac hypertrophy and heart failure, but safety concerns exist.

Other names

Carnitine O-palmitoyltransferase 1BM-CPT-1muscle isoform

Inhibition of CPT1b reduces fatty acid oxidation