期刊
NEUROSCIENCE
卷 166, 期 2, 页码 522-538出版社
PERGAMON-ELSEVIER SCIENCE LTD
DOI: 10.1016/j.neuroscience.2009.12.047
关键词
human myelin proteolipid protein; neurons; alternative splicing; Pelizaeus Merzbacher disease; spastic paraplegia type 2
资金
- ELA European Leukodystrophy Foundation
- INSERM
- Foundation for Medical Research [FRM ARS2000]
- Jean-Pierre and Nancy Boespflug myopathic research foundation
The human myelin proteolipid protein 1 gene (hPLP1), which encodes the major structural myelin proteins of the central nervous system (CNS), is classically described as expressed in the oligodendrocytes, the CNS myelinating cells. We identified two new exons in the intron 1 of the hPLP1 gene that lead to the expression of additional mRNA and protein isoforms mainly expressed in neurons instead of oligodendrocytes. Those novel neuronal PLP isoforms are detected as soon as human fetal development and their concomitant expression is specific of the human species. As classical PLP proteins, the novel protein isoforms seem to be addressed to the plasma membrane. These results suggest for the first time that PLP may have functions in humans not only in oligodendrocytes but also in neurons and could be implicated in axono-glial communication. Moreover, this neuronal expression of the hPLP1 gene might explain the neuronal dysfunctions in patients carrying hPLP1 gene mutations. (C) 2010 IBRO. Published by Elsevier Ltd. All rights reserved.
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