HMG-CoA Synthase exists as both a mitochondrial (mHMGCS) and cytoplasmic (cHMGCS) enzyme that condenses acetyl-CoA with acetoacetyl-CoA to form HMG-CoA. The HMG-CoA produced by cHMGCS is transformed into mevalonate by HMG-CoA reductase, which starts isoprenoid biosynthesis. End products of the isoprenoid pathway include cholesterol, ubiquinone, dolichol, isopentenyl adenosine and farnesyl groups. mHMGCS, together with HMG-CoA Lyase, is responsible for ketone body biosynthesis. mHMGCS is expressed in liver and kidney. Fasting, cAMP and fatty acids increase the level of transcription of mHMGCS, while feeding and insulin repress it. A regulatory element within the mHMGCS promoter confers transcriptional regulation by PPAR, RXR, COUP-TF and HNF-4.