TY - JOUR
T1 - The intralysosomal pH in cultured human skin fibroblasts in relation to cystine accumulation in patients with cystinosis
AU - Oude Elferink, R. P.
AU - Harms, E.
AU - Strijland, A.
AU - Tager, J. M.
PY - 1983
Y1 - 1983
N2 - The intralysosomal pH in cultured skin fibroblasts from a patient with cystinosis and from control fibroblasts was estimated by the method of Hollemans et al. (Hollemans, M., Oude Elferink, R.P.J., de Groot, P.G., Strijland, A. and Tager, J.M. (1981) Biochim. Biophys. Acta 643, 140-151). 1. The intralysosomal pH in cystinotic fibroblasts (5.37 +/- 0.04; n = 12) was almost identical to that in the control fibroblasts (5.27 +/- 0.06; n = 10). 2. After depletion of cystine by pretreatment of cystinotic fibroblasts with 1 mM cysteamine, there was no significant change in the intralysosomal pH. Incubation of either cystinotic or control fibroblasts with higher concentrations of cysteamine (10 or 25 mM) led to an increase in intralysosomal pH similar to that seen with other weak bases. 3. The fractional volume of the lysosomes in cystinotic fibroblasts (0.100 +/- 0.012; n = 4) was higher than that in control fibroblasts (0.039 +/- 0.010; n = 4). 4. It is concluded, in contrast to Jonas et al. (Jonas, A.J., Smith, M.L. and Schneider, J.A. (1982) J. Biol. Chem. 257, 13185-13188), that the lysosomal proton-translocating ATPase (Okhuma, S., Moriyama, Y. and Takano, T. (1982) Proc. Natl. Acad. Sci. USA 79, 2758-2762) is not impaired in cystinotic fibroblasts
AB - The intralysosomal pH in cultured skin fibroblasts from a patient with cystinosis and from control fibroblasts was estimated by the method of Hollemans et al. (Hollemans, M., Oude Elferink, R.P.J., de Groot, P.G., Strijland, A. and Tager, J.M. (1981) Biochim. Biophys. Acta 643, 140-151). 1. The intralysosomal pH in cystinotic fibroblasts (5.37 +/- 0.04; n = 12) was almost identical to that in the control fibroblasts (5.27 +/- 0.06; n = 10). 2. After depletion of cystine by pretreatment of cystinotic fibroblasts with 1 mM cysteamine, there was no significant change in the intralysosomal pH. Incubation of either cystinotic or control fibroblasts with higher concentrations of cysteamine (10 or 25 mM) led to an increase in intralysosomal pH similar to that seen with other weak bases. 3. The fractional volume of the lysosomes in cystinotic fibroblasts (0.100 +/- 0.012; n = 4) was higher than that in control fibroblasts (0.039 +/- 0.010; n = 4). 4. It is concluded, in contrast to Jonas et al. (Jonas, A.J., Smith, M.L. and Schneider, J.A. (1982) J. Biol. Chem. 257, 13185-13188), that the lysosomal proton-translocating ATPase (Okhuma, S., Moriyama, Y. and Takano, T. (1982) Proc. Natl. Acad. Sci. USA 79, 2758-2762) is not impaired in cystinotic fibroblasts
M3 - Article
C2 - 6639653
SN - 0006-291X
VL - 116
SP - 154
EP - 161
JO - Biochemical and Biophysical Research Communications
JF - Biochemical and Biophysical Research Communications
IS - 1
ER -