T
Toni L. Williamson
Researcher at University of California, San Diego
Publications - 7
Citations - 1277
Toni L. Williamson is an academic researcher from University of California, San Diego. The author has contributed to research in topics: Neurofilament & Motor neuron. The author has an hindex of 6, co-authored 7 publications receiving 1241 citations. Previous affiliations of Toni L. Williamson include Ludwig Institute for Cancer Research.
Papers
More filters
Journal ArticleDOI
Slowing of axonal transport is a very early event in the toxicity of ALS–linked SOD1 mutants to motor neurons
TL;DR: Reduced transport of selective cargoes of slow transport, especially tubulin, arises months before neurodegeneration in two SOD1 mutations linked to familial ALS, verifying the hypothesis that damage to the cargoe or machinery ofslow transport is an early feature of toxicity mediated by mutant SOD 1.
Journal ArticleDOI
Absence of neurofilaments reduces the selective vulnerability of motor neurons and slows disease caused by a familial amyotrophic lateral sclerosis-linked superoxide dismutase 1 mutant.
Toni L. Williamson,Lucie Bruijn,Qinzhang Zhu,Karen L. Anderson,Scott D. Anderson,Jean-Pierre Julien,Don W. Cleveland +6 more
TL;DR: By deleting NF-L, the major neurofilament subunit required for filament assembly, onset and progression of disease caused by familial ALS-linked SOD1 mutant G85R are significantly slowed, while selectivity of mutant-mediated toxicity for motor neurons is reduced.
Journal ArticleDOI
Neurofilament subunit NF-H modulates axonal diameter by selectively slowing neurofilament transport.
Joseph R. Marszalek,Toni L. Williamson,Michael K. Lee,Zuoshang Xu,Paul N. Hoffman,Mark W. Becher,Thomas O. Crawford,Don W. Cleveland +7 more
TL;DR: Mice constructed that express wild-type mouse NF-H up to 4.5 times the normal level are constructed to examine the mechanism through which neurofilaments regulate the caliber of myelinated axons and to test how aberrant accumulations of neurofilament cause motor neuron disease.
Journal ArticleDOI
Neurofilament-dependent radial growth of motor axons and axonal organization of neurofilaments does not require the neurofilament heavy subunit (NF-H) or its phosphorylation.
Mala V. Rao,Megan K. Houseweart,Toni L. Williamson,Thomas O. Crawford,Janet Folmer,Don W. Cleveland +5 more
TL;DR: Since postnatal growth of motor axon caliber continues largely unabated in the absence ofNF-H, neither interactions mediated by NF-H nor the extensive phosphorylation of it within myelinated axonal segments are essential features of this growth.
Journal ArticleDOI
Toxicity of ALS-linked SOD1 mutants.
Toni L. Williamson,Laura Corson,Lan Huang,Al Burlingame,Jian Liu,Lucie Bruijn,Don W. Cleveland +6 more
TL;DR: 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).