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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Veröffentlicht in:	PLoS genetics 2011-05, Vol.7 (5), p.e1002063-e1002063
Hauptverfasser:	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
Format:	Artikel
Sprache:	eng
Schlagworte:	Animals Base Sequence Biology Biosynthesis Brain Cell Differentiation Ceramides Ceramides - biosynthesis Ceramides - deficiency Cercopithecus aethiops COS Cells Degeneration Health aspects Homeostasis Lipofuscin - metabolism Membrane Proteins - genetics Membrane Proteins - metabolism Metabolic disorders Mice Mice, Inbred BALB C Mutation Nervous system Neurodegeneration Physiological aspects Proteins Purkinje cells Purkinje Cells - cytology Purkinje Cells - metabolism Sphingosine N-Acyltransferase - genetics Sphingosine N-Acyltransferase - metabolism
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