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Publication : Neurofilament homeostasis and motoneurone degeneration.

First Author  Perrone Capano C Year  2001
Journal  Bioessays Volume  23
Issue  1 Pages  24-33
PubMed ID  11135306 Mgi Jnum  J:66820
Mgi Id  MGI:1929318 Doi  10.1002/1521-1878(200101)23:1<24::AID-BIES1004>3.0.CO;2-H
Citation  Perrone Capano C, et al. (2001) Neurofilament homeostasis and motoneurone degeneration. Bioessays 23(1):24-33
abstractText  Neurofilament disorganisation is a hallmark of various neurodegenerative diseases. We review here current knowledge of neurofilament structure, gene expression and function. Neurofilament involvement in motoneurone neurological diseases is discussed in view of recent data from transgenic and spontaneous mouse mutants. In the mammalian neurone, the three neurofilament subunits are assembled into intermediate filaments as obligate heteropolymers. The subunits are expressed differentially during development and adult life according to the cell type and its physiological state. In addition to the well-established role of neurofilaments in the control of axonal calibre, there is increasing evidence that neurofilaments can interact with other cytoskeletal components and can modulate the axoplasmic flow. Although the extent to which neurofilament abnormalities contribute to the pathogenesis in human diseases remains unknown, emerging evidence suggests that disorganised neurofilaments can provoke degeneration and death of neurones. BioEssays 23:24-33, 2001. Copyright 2001 John Wiley & Sons, Inc.
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