A deficiency of ceramide biosynthesis causes cerebellar purkinje cell neurodegeneration and lipofuscin accumulation.

Sphingolipids, lipids with a common sphingoid base (also termed long chain base) backbone, play essential cellular structural and signaling functions. Alterations of sphingolipid levels have been implicated in many diseases, including neurodegenerative disorders. However, it remains largely unclear...

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Bibliographic Details
Main Authors: Lihong Zhao, Stefka D Spassieva, Thomas J Jucius, Leonard D Shultz, H Elizabeth Shick, Wendy B Macklin, Yusuf A Hannun, Lina M Obeid, Susan L Ackerman
Format: Article
Language:English
Published: Public Library of Science (PLoS) 2011-05-01
Series:PLoS Genetics
Online Access:http://europepmc.org/articles/PMC3098191?pdf=render