Protein Description
Isopentenyl-diphosphate delta-isomerase 1
Background
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.
Ligand Chemical Component
Acetic acid; Aminoethanolpyrophosphate; Magnesium ion; Manganese ion
Reference
Zhang, C., Liu, L., Xu, H., Wei, Z., Wang, Y., Lin, Y., Gong, W. (2007) Crystal structures of human IPP isomerase: new insights into the catalytic mechanism J.Mol.Biol. 366: 1437-1446