[Intravital determination of pH gradient between the cytoplasm and lysosomes in human fibroblasts in the normal state and in hereditary storage diseases].
Presented are the results of measurements of pH in cytoplasm and lysosomes of skin fibroblasts of healthy donors and patients with lysosomal storage diseases, mannosidosis, Fabry, Krabbe disease. The pH value was estimated in the stationary phase of growth using neutral red (lysosomes) and fluorescein diacetate (cytoplasm). It was shown that the cytoplasmic pH value in pathological cells didn't virtually differ from the control values. The intralysosomal pH value in fibroblasts of patients with mannosidosis and Fabry disease was essentially increased, which correlated with the size increase of these organelles upon the accumulation of unsplit compounds. This led to the decrease in pH gradient between the cytoplasm and lysosomes in the pathological cells, an increase in intralysosomal pH along with hereditary deficiency of enzymes could bring about the retardation of catabolic processes in lysosomes.
Ivleva
T S
TS
Ogloblina
T A
TA
Litinskaia
L L
LL
Khrust
Iu R
IuR
Vidershaĭn
G Ia
GIa
D002478:Cells, Cultured; D003593:Cytoplasm / Q000378:metabolism*; D000795:Fabry Disease / Q000378:metabolism; D005347:Fibroblasts / Q000378:metabolism; D006801:Humans; D006863:Hydrogen-Ion Concentration; D007965:Leukodystrophy, Globoid Cell / Q000378:metabolism; D008247:Lysosomes / Q000378:metabolism*; D008661:Metabolism, Inborn Errors / Q000378:metabolism*; D008363:alpha-Mannosidosis / Q000378:metabolism
Prizhiznennoe opredelenie gradienta pH mezhdu tsitoplasmoĭ i lizosomami v fibroblastakh cheloveka v norme i pri nasledstvennykh bolezniakh nakopleniia.