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Hsp70 stabilizes lysosomes and reverts Niemann–Pick disease-associated lysosomal pathology

Author

Listed:
  • Thomas Kirkegaard

    (Institute of Cancer Biology, Danish Cancer Society)

  • Anke G. Roth

    (Institute for Chemistry, Humboldt University)

  • Nikolaj H. T. Petersen

    (Institute of Cancer Biology, Danish Cancer Society)

  • Ajay K. Mahalka

    (Helsinki Biophysics & Biomembrane Group, Institute of Biomedicine, University of Helsinki)

  • Ole Dines Olsen

    (Institute of Cancer Biology, Danish Cancer Society
    University of Southern Denmark)

  • Irina Moilanen

    (Helsinki Biophysics & Biomembrane Group, Institute of Biomedicine, University of Helsinki)

  • Alicja Zylicz

    (International Institute of Molecular and Cell Biology)

  • Jens Knudsen

    (University of Southern Denmark)

  • Konrad Sandhoff

    (LIMES c/o Kekulé-Institute for Organic Chemistry and Biochemistry, University of Bonn)

  • Christoph Arenz

    (Institute for Chemistry, Humboldt University)

  • Paavo K. J. Kinnunen

    (Helsinki Biophysics & Biomembrane Group, Institute of Biomedicine, University of Helsinki)

  • Jesper Nylandsted

    (Institute of Cancer Biology, Danish Cancer Society)

  • Marja Jäättelä

    (Institute of Cancer Biology, Danish Cancer Society)

Abstract

Hsp70 rescues lysosomes The evolutionarily conserved chaperone protein known as Hsp70 (heat shock protein 70) promotes cell survival by inhibiting the permeabilization of lysosomal membranes. A new study of the mechanism of this process shows that localization of Hsp70 to lysosomes is crucial for its cytoprotective effect. Hsp70 binds to an anionic lipid called BMP, and this interaction results in stimulation of acid sphingomyelinase activity. Patients with Niemann–Pick disease, a severe lysosomal storage disorder, have decreased acid sphingomyelinase activity, and treatment of cells from such patients with recombinant Hsp70 is able to correct this phenotype and restore faulty lysosomes. This study provides a possible route to new treatments for lysosomal storage disorders.

Suggested Citation

  • Thomas Kirkegaard & Anke G. Roth & Nikolaj H. T. Petersen & Ajay K. Mahalka & Ole Dines Olsen & Irina Moilanen & Alicja Zylicz & Jens Knudsen & Konrad Sandhoff & Christoph Arenz & Paavo K. J. Kinnunen, 2010. "Hsp70 stabilizes lysosomes and reverts Niemann–Pick disease-associated lysosomal pathology," Nature, Nature, vol. 463(7280), pages 549-553, January.
    • Handle: RePEc:nat:nature:v:463:y:2010:i:7280:d:10.1038_nature08710
    DOI: 10.1038/nature08710
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