Fig. 1

Bempedoic acid and GSD1. Malonyl-CoA, which is synthesized from cytosolic acetyl-CoA, blocks the entry of long-chain fatty acids into the mitochondrial matrix by inhibition of CPT1. Bempedoyl-CoA inhibits ATP-citrate lyase. CAC, carnitine-acyl-carnitine exchanger; CPT, carnitine palmitoyltransferase; TCA, tricarboxylic acid cycle