Isoelectric Enzyme Patterns of Leukocyte Peroxidase in Normal Controls and Patients with Neuronal Ceroid-Lipofuscinoses

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Abstract
Using macro- and microanalytical isoelectric focusing techniques for separation of the soluble leukocytic peroxidase (hydrogen donor: p-phenylenediamine), 4 main isoenzyme components were found with pI [isoelectric point] at 9.6 (9.0), 7.6 (7.5), 6.2 (6.2) and 4.2 (4.7), which exhibited additional heterogeneities. The isoenzymes of the membrane-bound peroxidase displayed a similar pattern. The isoelectric subfractions of peroxidase in controls and patients with late-infantile (Jansky-Bielschowsky), juvenile (Spielmeyer-Sjogren) and adult (Kufs) types of neuronal ceroid-lipofuscinosis did not reveal any significant differences. Based on these findings, a deficiency of an isoenzyme component could not be held responsible for producing neuronal ceroid-lipofuscinoses, neither in patients with normal nor with reduced total activity of leukocyte peroxidase.

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