Accumulation of autophagic vacuoles and cardiomyopathy 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, Paul Saftig

研究成果: Contribution to journalArticle査読

636 被引用数 (Scopus)


Lysosome-associated membrane protein-2 (LAMP-2) is a highly glycosylated protein and an important constituent of the lysosomal membrane. 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 deficiency causing Danon's disease is associated with the accumulation of autophagic material in striated myocytes.

出版ステータス出版済み - 8 24 2000

All Science Journal Classification (ASJC) codes

  • General

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