Abstract
The acid proteinase and the peroxidase activity of peripheral leucocytes of respectively 12 and 13 patients suffering from Spielmeyer-Vogt's disease, alias Batten's disease, alias Stengel's disease was studied. Cathepsin D activity was found to be normal in the disease, but the cathepsin B activity was decreased by 24 percent. However, the decrease was insignificant. The peroxidase assay was made using Guajacol as well as p-phenylenediamine as substrate. When the assay was performed with p-phenylenediamine, the enzymic activities were significantly decreased (p less than or equal to 1%) compared to the activity of normal control leucocytes. Assaying peroxidase activity by means of Guajacol revealed a non-significant difference between the two groups. The data obtained indicate the syndrome under study to be due to hampered peroxidase activity. The defect found explains the occurrence of lipofuchsin in the nervous system previously demonstrated.
References
Jul 1, 1974·Cancer·M Catterall
Feb 15, 1974·Brain Research·K R Govindarajan, J Clausen
Sep 1, 1971·Experimental Eye Research·E M BurdenJ Chayen
Nov 1, 1962·Journal of Neurology, Neurosurgery, and Psychiatry·W ZEMAN, J HOFFMAN
Jun 3, 1960·Science·F Seitelberger
Citations
Mar 13, 1978·European Journal of Pediatrics·C M Plum, A Nielsen
Sep 1, 1978·Journal of the Neurological Sciences·W R Den Tandt, J J Martin
Mar 1, 1978·Neurology India·K RadhakrishnanJ S Chopra
Aug 1, 1978·Journal of Neurochemistry·B Schwerer, H Bernheimer
Aug 1, 1978·Journal of Neurochemistry·S Marklund, C M Plum
Jan 1, 1988·American Journal of Medical Genetics. Supplement·L Black, R K Pullarkat
Apr 1, 1977·Scandinavian Journal of Clinical and Laboratory Investigation·A Brock
Dec 1, 1977·Acta Neurologica Scandinavica·S BrandtJ C Melchior
Dec 1, 1977·Archives internationales de physiologie et de biochimie· De WolfW Dierick
Jun 1, 1978·Scandinavian Journal of Clinical and Laboratory Investigation·G E JensenJ Clausen
Feb 1, 1977·Developmental Medicine and Child Neurology·N Z BaquerA L Greenbaum
Mar 1, 1978·Journal of the Neurological Sciences·M F TsanP A McIntyre
Jul 1, 1978·Meat Science·B Venugopal, M E Bailey