Your browser does not support javascript:   Search for gard hereSearch for news-and-events here.


Genetic and Rare Diseases Information Center (GARD)

Print friendly version

Juvenile primary lateral sclerosis

Other Names for this Disease
  • JPLS
  • PLS juvenile
  • Primary lateral sclerosis, juvenile
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.


Newline Maker

What causes juvenile primary lateral sclerosis?

Juvenile primary lateral sclerosis is caused by mutations in the ALS2 gene. The ALS2 gene provides instructions for making a protein called alsin. Alsin is abundant in motor neurons, but its function is not fully understood. Mutations in the ALS2 gene alter the instructions for producing alsin. As a result, alsin is unstable and decays rapidly, or it is disabled and cannot function properly. It is unclear how the loss of functional alsin protein damages motor neurons and causes juvenile primary lateral sclerosis.[1]
Last updated: 3/4/2011

  1. Juvenile primary lateral sclerosis. Genetics Home Reference (GHR). 2007; Accessed 3/4/2011.