Neurodegeneration Induced by De-Regulation of Neurofilament Gene Expression in Transgenic Mice
Neurofilaments (NFs) are formed by the copolymerization of three proteins, the light (NF-L), medium (NF-M) and heavy (NF-H) subunits (Hoffman and Lasek,1975; Liem et al., 1978). The three NF subunits are encoded by different genes and several reports have shown that the genes are under separate developmental control (Shaw and Weber, 1982; Carden et al., 1987; Cochard and Paulin, 1984; Julien et al., 1986). Direct evidence for a role of neurofilaments in the control of axonal calibers was recently provided from the recent analyses of a mutant quail (Ohara et al. 1993) and of a transgenic mouse (Eyer and Peterson 1994) with NF-deficient axons.
KeywordsAmyotrophic Lateral Sclerosis Axonal Transport Amyotrophic Lateral Sclerosis Patient Motor Neuron Disease Spinal Motor Neuron
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