Article
A deficiency of ceramide biosynthesis causes cerebellar purkinje cell neurodegeneration and lipofuscin accumulation.
PLoS genetics - 1 May 2011
Zhao Lihong, Spassieva Stefka D, Jucius Thomas J, Shultz Leonard D, Shick H Elizabeth, Macklin Wendy B, Hannun Yusuf A, Obeid Lina M, Ackerman Susan L
Abstract excerpt
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 whether sphingolipid changes in these diseases are pathological events or homeostatic responses. Furthermore, how...
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