Accumulation of autophagic vacuoles and cardiomyopathy in LAMP-2-deficient mice
- Y Tanaka 1, G Guhde , A Suter , E L Eskelinen , D Hartmann , R Lüllmann-Rauch , P M Janssen , J Blanz , K von Figura , P Saftig
- 1Zentrum Biochemie und Molekulare Zellbiologie, Abt. Biochemie II, Universität Göttingen, Germany.
- 0Zentrum Biochemie und Molekulare Zellbiologie, Abt. Biochemie II, Universität Göttingen, Germany.
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View abstract on PubMed
Summary
This summary is machine-generated.Lysosome-associated membrane protein-2 (LAMP-2) deficiency in mice causes early mortality and impaired autophagy, leading to autophagic vacuoles accumulation. This highlights LAMP-2
Area Of Science
- Cell Biology
- Molecular Biology
- Genetics
Background
- Lysosome-associated membrane protein-2 (LAMP-2) is a key lysosomal membrane protein.
- LAMP-2 plays a role in cellular degradation processes.
Purpose Of The Study
- To investigate the function of LAMP-2 in autophagy.
- To determine the consequences of LAMP-2 deficiency in vivo.
Main Methods
- Generation and analysis of LAMP-2 deficient mice.
- Ultrastructural examination of tissues from deficient mice.
- Assessment of protein degradation in hepatocytes.
Main Results
- LAMP-2 deficiency led to increased mortality in mice (20-40 days).
- Accumulation of autophagic vacuoles observed in multiple tissues.
- Impaired autophagic degradation of long-lived proteins in hepatocytes.
- Cardiac myocyte abnormalities and reduced heart contractility.
Conclusions
- LAMP-2 is critical for the proper functioning of autophagy.
- LAMP-2 deficiency results in severe autophagic defects and organ dysfunction.
- Findings are consistent with human Danon's disease, linked to LAMP-2 deficiency.
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