Results 31 to 40 of about 1,397,252 (312)

A Novel Calpain Inhibitor Compound Has Protective Effects on a Zebrafish Model of Spinocerebellar Ataxia Type 3

open access: yesCells, 2021
Spinocerebellar ataxia type 3 (SCA3) is a hereditary ataxia caused by inheritance of a mutated form of the human ATXN3 gene containing an expanded CAG repeat region, encoding a human ataxin-3 protein with a long polyglutamine (polyQ) repeat region ...
Katherine J. Robinson   +4 more
doaj   +1 more source

Motor Neurone Disease [PDF]

open access: yesPostgraduate Medical Journal, 1962
THE story of motor neurone disease goes back more than a hundred years, to the latter half of the igth century, and the days of the great clinical neurologists of France. Of the many famous names linked to this story three are pre-eminent: Charcot-physician and neuropathologist, and great teacher, who became even more renowned for his studies of ...
openaire   +2 more sources

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

open access: yes, 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.   +6 more
core   +1 more source

Neural basis of a visuo-motor transformation in the fly [PDF]

open access: yes, 2009
How the outputs of populations of sensory neurons are used by motor systems to generate appropriate behaviour is a long standing question in neuroscience. I address this problem by studying a comparatively simple model system.

core   +2 more sources

Unbiased Label-Free Quantitative Proteomics of Cells Expressing Amyotrophic Lateral Sclerosis (ALS) Mutations in CCNF Reveals Activation of the Apoptosis Pathway: A Workflow to Screen Pathogenic Gene Mutations

open access: yesFrontiers in Molecular Neuroscience, 2021
The past decade has seen a rapid acceleration in the discovery of new genetic causes of ALS, with more than 20 putative ALS-causing genes now cited. These genes encode proteins that cover a diverse range of molecular functions, including free radical ...
Flora Cheng   +28 more
doaj   +1 more source

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

open access: yes, 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   +33 more
core   +1 more source

Motor neurone disease [PDF]

open access: yesPostgraduate Medical Journal, 2002
Abstract Motor neurone disease (MND), or amyotrophic lateral sclerosis (ALS), is a neurodegenerative disorder of unknown aetiology. Progressive motor weakness and bulbar dysfunction lead to premature death, usually from respiratory failure. Confirming the diagnosis may initially be difficult until the full clinical features are manifest.
openaire   +3 more sources

Motor Band Sign in Motor Neuron Disease: A Marker for Upper Motor Neuron Involvement

open access: yesCanadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques, 2022
ABSTRACT:Background and Objective:The prevalence and role of the motor band sign (MBS) remain unclear in motor neuron disease. We report the frequency of MBS in amyotrophic lateral sclerosis (ALS) and primary lateral sclerosis (PLS), its correlation with clinical upper motor neuron (UMN) signs, and prognostic value in ALS.Methods:We conducted a ...
Hee Sahng Chung   +3 more
openaire   +2 more sources

cDNA that encodes active agrin [PDF]

open access: yes, 1992
Agrin is thought to mediate the motor neuron-induced aggregation of AChRs and AChE on the surface of muscle fibers at neuromuscular junctions. We have isolated a cDNA from a chick brain library that, based on sequence homology and expression experiments,
Kröger, Stephan   +9 more
core   +1 more source

Real-time visualization of oxidative stress-mediated neurodegeneration of individual spinal motor neurons in vivo

open access: yesRedox Biology, 2018
Generation of reactive oxygen species (ROS) has been shown to be important for many physiological processes, ranging from cell differentiation to apoptosis. With the development of the genetically encoded photosensitiser KillerRed (KR) it is now possible
Isabel Formella   +8 more
doaj   +1 more source

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