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The isoprene biosynthetic pathway supply the cell with cholesterol, ubiquinone, and various nonsterol metabolites. The farnesylpyrophosphate synthetase enzyme catalyzes the formation of geranyl and farnesylpyrophosphate from isopentenylpyrophosphate and dimethylallyl pyrophosphate. Analysis of FDPS activity and protein in rat liver, accompanied by immunofluorescence and immunoelectron microscopy studies, demonstrated that FDPS is predominantly localized in peroxisomes. 1 Liver tissue from patients with the peroxisomal deficiency diseases Zellweger syndrome and neonatal adrenoleukodystrophy exhibit diminished activities of FDPS and subsequent isoprenoid synthesis.
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