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- W2010622543 abstract "The biochemical lesion in cystinotic cells resulting in lysosomal storage of cystine is not yet known. The lysosomal metabolism of cystine should be investigated in isolated lysosomes. In whole unfractionated cells, the cystine metabolism was found to be unchanged /Patrick 1962, Schneider et al. 1967/. Human skin fibroblasts were cultured in large scale /2 × 108 to 1 × 109 cells in one batch/. After differential centrifugation free-flow electrophoresis according to the method of Hannig et al. was used for isolating native lysosomes from these fibroblasts. The purity of lysosomal fractions was controlled by electron microscopy and by measuring enzyme markers for lysosomes, mitochondria and peroxisomes. The activity of enzymes involved in cystine reduction in cystinotic lysosomes was compared to that in normal lysosomes /cystine-glutathione-transhydrogenase, glutathione-reductase/." @default.
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- W2010622543 date "1975-11-01" @default.
- W2010622543 modified "2023-09-26" @default.
- W2010622543 title "ISOLATION OF LYSOSOMES FROM SKIN FIBROBLASTS OF NORMAL AND CYSTINOTIC INVIDUALS" @default.
- W2010622543 doi "https://doi.org/10.1203/00006450-197511000-00046" @default.
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