Results from electron microscopic morphometry,
enzyme cytochemistry and immunolocalization in liver biopsies are reviewed. Emphasis is put on the following aspects: 1) relationship between peroxisomal size and
enzyme concentration; 2) abnormal enlargement of peroxisomes in many
congenital disorders with peroxisomal dysfunction; 3) normal localization of matrix
enzymes in several patients with peroxisomal dysfunction, with the exception of
catalase, which is mainly cytoplasmic; 4) ghost-like peroxisomes in the liver of several syndromes but not in nine cases labelled as Zellweger; 5) discrepancies between liver and cultured fibroblasts; 6) trilamellar, regularly spaced inclusions, large stacks of which are birefringent, indicate a peroxisomal dysfunction; their absence does not exclude it. The same rule holds for
lipid in macrophages which is insoluble in
acetone and
n-hexane (after fixation). The chemical nature of these two storage materials remains unclear; and 7) proliferation of human peroxisomes is frequent in acquired
liver diseases and
drug toxicity, but is never accompanied by an increase in size, in contrast to the effect of the
fibrates and phthalates in rat and mouse. Novel data from seven peroxisomal patients are included.