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Related Experiment Videos

Elevated lysosomal pH in Mucolipidosis type IV cells.

G Bach1, C S Chen, R E Pagano

  • 1Department of Human Genetics, Hadassah University Hospital, Jerusalem, Israel. bach@hadassah.org.il

Clinica Chimica Acta; International Journal of Clinical Chemistry
|March 25, 1999
PubMed
Summary

Lysosomal pH is elevated in Mucolipidosis type IV (ML-IV) cells, unlike other storage diseases. This unique characteristic may explain the accumulation of substances in ML-IV cells.

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Area of Science:

  • Cell Biology
  • Biochemistry
  • Genetics

Background:

  • Lysosomal storage diseases (LSDs) involve the accumulation of undegraded substances within lysosomes.
  • The intralysosomal pH is crucial for the function of lysosomal hydrolases.
  • Mucolipidosis type IV (ML-IV) is an LSD with poorly understood pathogenesis.

Purpose of the Study:

  • To determine the lysosomal pH in ML-IV fibroblasts compared to other LSDs and normal fibroblasts.
  • To investigate if elevated lysosomal pH is a unique feature of ML-IV.

Main Methods:

  • In situ measurement of lysosomal pH in living human fibroblasts using fluorescein-conjugated dextran and quantitative fluorescence microscopy.
  • Ratiometric fluorescence measurements converted to absolute pH values using a standard curve.

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  • Validation of results using acridine orange, a pH-sensitive fluorophore.

    • Main Results:

    • Lysosomal pH in normal and other LSD fibroblasts (Niemann Pick A/C, Hunter, Farber) ranged from 4.3 to 4.5.
    • ML-IV fibroblasts exhibited a significantly higher lysosomal pH, approximately 5.2.
    • Results were consistent when using acridine orange, excluding measurement artifacts.

    Conclusions:

    • Elevated lysosomal pH is a unique characteristic of ML-IV cells.
    • This unique defect in lysosomal pH may be a key factor, or the cause, of substrate accumulation in ML-IV.
    • Despite the altered pH, lysosomal hydrolases in ML-IV cells appear to be functionally normal.