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

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, Jesper Nylandsted and Marja Jäättelä ()
Additional contact information
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
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

Nature, 2010, vol. 463, issue 7280, 549-553

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.

Date: 2010
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DOI: 10.1038/nature08710

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