Elevated lysosomal pH in neuronal ceroid lipofuscinoses (NCLs).

  title={Elevated lysosomal pH in neuronal ceroid lipofuscinoses (NCLs).},
  author={Juha Holopainen and Juhani Saarikoski and Paavo K. J. Kinnunen and Irma E. J{\"a}rvel{\"a}},
  journal={European journal of biochemistry},
  volume={268 22},
We report here the intracellular (pHi) and lysosomal pH in fibroblasts of six forms of neuronal ceroid lipofuscinoses (NCLs). Acid extrusion rate and pH(i) values were measured by the membrane-permeant acetoxymethyl ester of the indicator dye, 2',7'-bis(carboxyethyl)-5-(and-6)-carboxy-fluorescein (BCECF) and lysosomal pH by a spectrofluorometric assay utilizing a novel acidotropic probe, Lysosensor yellow/blue. Intracellular pH was normal in all NCLs. Elevated lysosomal pH was detected in all… CONTINUE READING


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