What is the role of lysosome-associated membrane protein 2 (LAMP2) in the etiology of Wolff-Parkinson-White (WPW) syndrome?

Updated: Jan 08, 2017
  • Author: Christopher R Ellis, MD, FACC, FHRS; Chief Editor: Mikhael F El-Chami, MD  more...
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Answer

Mutations in the lysosome-associated membrane protein 2 (LAMP2), which cause accumulation of cardiac glycogen, are thought to be the etiology of a significant number of hypertrophic cardiomyopathies in children, especially when skeletal myopathy, WPW syndrome, or both are present.

For example, Danon disease is an X-linked lysosomal cardioskeletal myopathy; males are more often and more severely affected than females. It is caused by mutations in the LAMP2 that produce proximal muscle weakness and mild atrophy, left ventricle hypertrophy, WPW syndrome, and mental retardation.


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