Toni L. Williamson

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Neurofilaments are essential for establishment and maintenance of axonal diameter of large myelinated axons, a property that determines the velocity of electrical signal conduction. One prominent model for how neurofilaments specify axonal growth is that the 660-amino acid, heavily phosphorylated tail domain of neurofilament heavy subunit (NF-H) is(More)
Alpha-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid (AMPA) receptor antagonists have been shown to have neuroprotective effects in stroke models and although clinical trials with some agents are still ongoing, published results have not been favourable. We therefore wished to compare the effects of GYKI 52466, GYKI 53405, EGIS-8332 and EGIS-10608,(More)
The efficacy of gamma-secretase inhibitors in vivo has, to date, been generally assessed in transgenic mouse models expressing increased levels of amyloid-beta (Abeta) peptide thereby allowing the detection of changes in Abeta production. However, it is not clear whether the in vivo potency of gamma-secretase inhibitors is independent of the level of(More)
An important problem in speech perception is to determine how humans extract the perceptually invariant place of articulation information in the speech wave across variable acoustic contexts. Although analyses have been developed that attempted to classify the voiced stops /b/ versus /d/ from stimulus onset information, most of the human perceptual research(More)
Absolute thresholds for complex vowel stimuli were compared in Mongolian gerbils (Meriones unguiculatus) as a function of age and genetic origin. For a group of 12-month-old 'domestic' gerbils obtained from Tumblebrook Farms, lowest thresholds averaging 14 dB SPL occurred for the vowel /alpha/, which had its most intense formant (F1) at 730 Hz. Thresholds(More)
Genome-wide gene expression profiling has become standard for assessing potential liabilities as well as for elucidating mechanisms of toxicity of drug candidates under development. Analysis of microarray data is often challenging due to the lack of a statistical model that is amenable to biological variation in a small number of samples. Here we present a(More)
50 nature neuroscience • volume 2 no 1 • january 1999 articles ALS is a progressive neurodegenerative disease occurring in middle to late life and characterized by the loss of large motor neurons of the spinal cord, brain stem and motor cortex. Dysfunction and death of these motor neurons causes muscle weakness and atrophy leading to paralysis and death in(More)
Estévez et al. (1) focused on a potential mechanism through which dominantly inherited mutation in superoxide dismutase 1 (SOD1), an abundant, ubiquitously expressed antioxidant protein, triggers the selective death of motor neurons in amyotrophic lateral sclerosis (ALS). Each subunit of SOD1 binds one zinc and one copper atom. Dismutation of the superoxide(More)
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