Accumulation of autophagic vacuoles and cardiomyopathy in LAMP-2-deficient mice

Yoshitaka Tanaka, Gundula Guhde, Anke Suter, Eeva-Liisa Eskelinen, Dieter Hartmann, Renate Lüllmann-Rauch, Paul M. L. Janssen, Judith Blanz, Kurt von Figura and Paul Saftig ()
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Yoshitaka Tanaka: Zentrum Biochemie und Molekulare Zellbiologie, Abt. Biochemie II, Universität Göttingen, Heinrich-Düker-Weg 12
Gundula Guhde: Zentrum Biochemie und Molekulare Zellbiologie, Abt. Biochemie II, Universität Göttingen, Heinrich-Düker-Weg 12
Anke Suter: Zentrum Biochemie und Molekulare Zellbiologie, Abt. Biochemie II, Universität Göttingen, Heinrich-Düker-Weg 12
Eeva-Liisa Eskelinen: University of Dundee
Dieter Hartmann: Anatomisches Institut, Christian Albrechts Universität Kiel
Renate Lüllmann-Rauch: Anatomisches Institut, Christian Albrechts Universität Kiel
Paul M. L. Janssen: Abteilung Kardiologie und Pneumologie Universität Göttingen
Judith Blanz: Zentrum Biochemie und Molekulare Zellbiologie, Abt. Biochemie II, Universität Göttingen, Heinrich-Düker-Weg 12
Kurt von Figura: Zentrum Biochemie und Molekulare Zellbiologie, Abt. Biochemie II, Universität Göttingen, Heinrich-Düker-Weg 12
Paul Saftig: Zentrum Biochemie und Molekulare Zellbiologie, Abt. Biochemie II, Universität Göttingen, Heinrich-Düker-Weg 12

Nature, 2000, vol. 406, issue 6798, 902-906

Abstract: Abstract Lysosome-associated membrane protein-2 (LAMP-2) is a highly glycosylated protein and an important constituent of the lysosomal membrane1,2,3,4,5,6,7. Here we show that LAMP-2 deficiency in mice increases mortality between 20 and 40 days of age. The surviving mice are fertile and have an almost normal life span. Ultrastructurally, there is extensive accumulation of autophagic vacuoles in many tissues including liver, pancreas, spleen, kidney and skeletal and heart muscle. In hepatocytes, the autophagic degradation of long-lived proteins is severely impaired. Cardiac myocytes are ultrastructurally abnormal and heart contractility is severely reduced. These findings indicate that LAMP-2 is critical for autophagy. This theory is further substantiated by the finding that human LAMP-2 deficiency8 causing Danon's disease is associated with the accumulation of autophagic material in striated myocytes.

Date: 2000
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DOI: 10.1038/35022595

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