A mitochondrial ketogenic enzyme regulates its gene expression by association with the nuclear hormone receptor PPARα

Mitochondrial 3‐hydroxy‐3‐methylglutaryl‐CoA synthase (mHMG‐CoAS) is a key enzyme in ketogenesis, catalyzing the condensation of acetyl‐CoA and acetoacetyl‐CoA to generate HMG‐CoA, which is eventually converted to ketone bodies. Transcription of the nuclear‐encoded gene for mHMG‐CoAS is stimulated by peroxisome proliferator‐activated receptor (PPAR) α, a fatty acid‐activated nuclear hormone receptor. Here we show that the mHMG‐CoAS protein …