Isopentenyl Diphosphate Delta Isomerase 1 (IDI1)

IDI1 encodes a peroxisomally-localized enzyme that catalyzes the interconversion of isopentenyl diphosphate (IPP) to its highly electrophilic isomer, dimethylallyl diphosphate (DMAPP), which are the substrates for the successive reaction that results in the synthesis of farnesyl diphosphate and, ultimately, cholesterol. It has been shown in peroxisomal deficiency diseases such as Zellweger syndrome and neonatal adrenoleukodystrophy that there is reduction in IPP isomerase activity. The predicted 228-amino acid human protein shares 50% identity with S. cerevisiae IPP isomerase, although the human protein is approximately 65 amino acids shorter at the N terminus.