Results 31 to 40 of about 6,008,570 (306)

The management of motor neurone disease [PDF]

Journal of Neurology, Neurosurgery & Psychiatry, 2003
The management of motor neurone disease (MND) has evolved rapidly over the last two decades. Although still incurable, MND is not untreatable. From an attitude of nihilism, treatments and interventions that prolong survival have been developed. These treatments do not, however, arrest progression or reverse weakness.
Leigh, P N, Abrahams, S, Al-Chalabi, A, Ampong, M A, Goldstein, L H, Johnson, J, Lyall, R, Moxham, J, Mustfa, N, Rio, A, Shaw, C, Willey, E   +11 more
openaire   +2 more sources

Inflammation in ALS and SMA: Sorting out the good from the evil

Neurobiology of Disease, 2010
Indices of neuroinflammation are found in a variety of diseases of the CNS including amyotrophic lateral sclerosis (ALS) and spinal muscular atrophy (SMA).
Dimitra Papadimitriou, Virginia Le Verche, Arnaud Jacquier, Burcin Ikiz, Serge Przedborski, Diane B. Re   +5 more
doaj   +1 more source

ALS-associated mutant FUS induces selective motor neuron degeneration through toxic gain of function

Nature Communications, 2016
The mechanism by which FUS mutations cause familial ALS remains unclear. Here, the authors use mouse transgenic models to show that a toxic gain-of-function underlies motor neuron degeneration, and that the toxicity of mutant FUS does not depend on a ...
Aarti Sharma, Alexander K. Lyashchenko, Lei Lu, Sara Ebrahimi Nasrabady, Margot Elmaleh, Monica Mendelsohn, Adriana Nemes, Juan Carlos Tapia, George Z. Mentis, Neil A. Shneider   +9 more
doaj   +1 more source

Telehealth for motor neurone disease [PDF]

Medical Journal of Australia, 2014
Tᴏ ᴛʜᴇ Eᴅɪᴛᴏʀ: Telehealth is an expanding area with emerging evidence of use in the management of motor neurone disease (MND) and home mechanical ventilation. Here, we report our experience with the use of telehealth for managing MND in Queensland.
Henderson, Robert D., Hutchinson, Nicole, Douglas, James A., Douglas, Carol   +3 more
openaire   +5 more sources

Slow motor neuron stimulation of locust skeletal muscle: model and measurement

, 2013
The isometric force response of the locust hind leg extensor tibia muscle to stimulation of a slow extensor tibia motor neuron is experimentally investigated, and a mathematical model describing the response presented.
Mace, Brian R., Rustighi, Emiliano, Wilson, Emma, Mace, Brian R, Newland, Philip L, Newland, Philip L., Wilson, Emma Denise   +6 more
core   +1 more source

Mutant glycyl-tRNA synthetase (Gars) ameliorates SOD1G93A motor neuron degeneration phenotype but has little affect on Loa dynein heavy chain mutant mice [PDF]

, 2009
Background: In humans, mutations in the enzyme glycyl-tRNA synthetase (GARS) cause motor and sensory axon loss in the peripheral nervous system, and clinical phenotypes ranging from Charcot-Marie-Tooth neuropathy to a severe infantile form of spinal ...
Hazel P Williams, Greensmith Linda, Francesca Achilli, Ruth Chia, Elizabeth M. C. Fisher, Hazel P. Williams, Fisher Elizabeth M. C., Achilli, F., Chia, R, Bryson, J.B., Bros-Facer, V., Greensmith, L., Bros-Facer, V, Banks, G.T., Bryson, JB, Banks, GT, Fisher, EMC, Bros-Facer Virginie, Linda Greensmith, Fisher, E.M.C., Banks Gareth T., Chia, R., Gareth T Banks, Williams, HP, Greensmith, L, Williams Hazel P., Elizabeth M C Fisher, J Barney Bryson, Gareth T. Banks, Virginie Bros-Facer, Achilli Francesca, Williams, H.P., Bryson J. Barney, Chia Ruth, J. Barney Bryson, Achilli, F   +35 more
core   +1 more source

Long-term muscle-specific overexpression of DOK7 in mice using AAV9-tMCK-DOK7

Molecular Therapy: Nucleic Acids, 2023
Neuromuscular junction (NMJ) dysfunction underlies several diseases, including congenital myasthenic syndromes (CMSs) and motor neuron disease (MND).
Yu-Ting Huang, Hannah R. Crick, Helena Chaytow, Dinja van der Hoorn, Abrar Alhindi, Ross A. Jones, Ralph D. Hector, Stuart R. Cobb, Thomas H. Gillingwater   +8 more
doaj   +1 more source

Spinal muscular atrophy patient iPSC-derived motor neurons have reduced expression of proteins important in neuronal development [PDF]

, 2016
This work was supported by The RJAH Institute of Orthopaedics, UK (H.F.), The SMA Trust, UK (H.F.), Cedars-Sinai Institutional startup funds (D.S), California Institute for Regenerative Medicine Grant RT-02040 (D.S.), National Center for Advancing ...
Heidi R. Fuller, Andrew R. Gross, Kaus, Anjoscha, Morris, Glen, Sareen, Dhruv, Glenn E. Morris, Fuller, H, Shirran, Sally Lorna, Catherine H. Botting, Shirran, SL, Botting, Catherine Helen, Dhruv Sareen, Mandefro, Berhan, Botting, CH, Gross, AR, Berhan Mandefro, Fuller, Heidi, Sally L. Shirran, Sareen, S, Morris, GE, Anjoscha S. Kaus, Gross, Andrew, Mandefro, B, Kaus, AS   +23 more
core   +1 more source

Decoding the molecular mechanism underpinning motor neuron degeneration in ALS

, 2023
reservedMotor neuron degeneration is one of the main feature of different neuromuscular diseases, including Amyotrophic Lateral Sclerosis (ALS). The molecular mechanism underpinning motor neuron degeneration has not been well understood yet, hampering ...
SARTORE, LUIGI
core  

Plasma Neurofilament Heavy Chain Levels Correlate to Markers of Late Stage Disease Progression and Treatment Response in SOD1(G93A) Mice that Model ALS [PDF]

, 2012
Background: Amyotrophic lateral sclerosis (ALS) is an incurable neurodegenerative disorder characterised by progressive degeneration of motor neurons leading to death, typically within 3–5 years of symptom onset. The diagnosis of ALS is largely reliant
Greensmith Linda, Dick, J., Lu, C.H., Malaspina, A., Kalmar, B., Greensmith, L., Dick, J, Petzold, A.F.S., Petzold, A, Linda Greensmith, Malaspina Andrea, Petzold Axel, Greensmith, L, Kalmar Bernadett, Kalmar, B, Dick James, Lu Ching-Hua, Lu, CH, Bernadett Kalmar, James Dick, Ching-Hua Lu, Lu, C-H, Andrea Malaspina, Malaspina, A, Axel Petzold   +24 more
core   +3 more sources