Preparing your results

Our searching services are busy right now. Your search will reload in five seconds.

X
Forgot Password

If you have forgotten your password you can enter your email here and get a temporary password sent to your email.

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

Nature | Aug 24, 2000

http://www.ncbi.nlm.nih.gov/pubmed/10972293

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.

Pubmed ID: 10972293 RIS Download

Mesh terms: Amino Acids | Animals | Antigens, CD | Autophagy | Body Weight | Cardiomyopathies | Cells, Cultured | Crosses, Genetic | Female | Gene Targeting | Glucagon | Humans | Liver | Lysosomal Storage Diseases | Lysosome-Associated Membrane Glycoproteins | Male | Membrane Glycoproteins | Mice | Muscles | Myocardial Contraction | Organ Size | Pancreas | Vacuoles

Research resources used in this publication

None found

Research tools detected in this publication

None found

Data used in this publication

None found

Associated grants

None

Mouse Genome Informatics (Data, Gene Annotation)

Publication data is provided by the National Library of Medicine ® and PubMed ®. Data is retrieved from PubMed ® on a weekly schedule. For terms and conditions see the National Library of Medicine Terms and Conditions.