Abstract
Myocardium from English setters affected with ceroid lipofuscinosis (CCL) was maintained in culture for up to 14 days. Supplementing the culture medium with 0.2 mg/ml of N-acetyl-DL-homocysteine thiolactone (AHT) or with 5 μgml of dimethylaminoethyl-p-chlorophenoxyacetic acid (DPX) caused a significant reduction in number and an increase in size and volume density of lipopigment granules as determined by computer morphometric analysis. Electron microscopy showed changes in the consistency of the lipopigment in the form of vacuoles and pigment-body fragments. The antioxidant nordihydroguaiaretic acid (NDGA) caused an increase in number, size and volume density of the pigment granules. NN′-diphenyl-p-phenylenediamine (DPPD) and the enzyme horseradish peroxidase (HRP) may also produce similar changes when concentrations lower than 10 μg/ml and 80 μg/ml respectively are being used.
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Spoerri, P.E., Eins, S., Kelley, K. et al. Ceroid lipopigment: A morphometric study on its breakdown. AGE 8, 58–63 (1985). https://doi.org/10.1007/BF02432072
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DOI: https://doi.org/10.1007/BF02432072