液泡
自噬
收缩性
心肌细胞
溶酶体
细胞生物学
骨骼肌
心肌病
生物
内分泌学
心肌
内科学
胰腺
化学
心力衰竭
医学
细胞质
生物化学
细胞凋亡
酶
作者
Yoshitaka Tanaka,Gundula Guhde,A. Suter,Eeva‐Liisa Eskelinen,Dieter Hartmann,Renate Lüllmann‐Rauch,Paul M. L. Janssen,Judith Blanz,Kurt Von Figura,Paul Säftig
出处
期刊:Nature
[Springer Nature]
日期:2000-08-01
卷期号:406 (6798): 902-906
被引量:816
摘要
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.
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