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• W2082649761 abstract "Extract: Continuous leukocyte cultures were established from the blood of three siblings with classical nephropathic cystinosis and from their mother. The cells from patients with cystinosis (CC) contained at least 15− to 20-fold more cystine than cultured leukocytes from normal individuals (NC). The transport of cystine-35S into CC was similar to uptake into NC when tested at pH 5.0 and pH 7.4. The cultured CC could not be distinguished by ultrastructure from NC Crystalline inclusions which could easily have been mistaken for cystine were observed in the lysosome of a normal cell. The modal number of chromosomes in CC and NC lines was 46. There was a marked degree of aneuploidy. Tetraploidy was present in 3.8–9.6% of metaphases. Structural rearrangements and breaks were seen in all cell lines. Speculation: Continuous leukocyte cultures may prove a useful experimental tool for further studies of the basic metabolic derangement responsible for abnormal intracellular accumulations of cystine in cystinotic cells (CC). The cultures are a convenient source of relatively large amounts of pure cellular material for investigative use. Because they grow in suspension, cultured leukocytes are well suited for investigation of transport phenomena in normal human cells (NC) as well as those with genetic diseases. The absence of a defect in the uptake of cystine at pH 5.0 in CC fails to support the hypothesis of a lysosomal transport abnormality for cystine in these cells, yet cannot be regarded as firm refutation of that hypothesis. Elucidation of the basic metabolic defect in cystinosis may require studies of the enzymology and transport systems of isolated lysosomes; such transport studies may have formidable technical problems. The demonstration of intralysosomal crystals which could easily have been mistaken for cystine in one line of cultured NC emphasizes the need for great caution in assigning pathophysiologic significance to any rare crystalline bodies seen only in occasional lysosomes or other organelles of CC. The chromosome abnormalities, and perhaps the capacity to proliferate, of continuously cultured leukocytes may be related to infection with herpes-like virus, as has been previously suggested." @default.
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• W2082649761 date "1971-10-01" @default.
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• W2082649761 title "Biochemical, Morphologic, and Gytogenetic Studies of Leukocytes Growing in Continuous Culture from Normal Individuals and from Patients with Cystinosis" @default.
• W2082649761 doi "https://doi.org/10.1203/00006450-197110000-00001" @default.
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