نتایج جستجو برای: motor neuron disorder

تعداد نتایج: 774054  

Journal: :The Journal of pharmacology and experimental therapeutics 2007
Thierry Bordet Bruno Buisson Magali Michaud Cyrille Drouot Pascale Galéa Pierre Delaage Natalia P Akentieva Alex S Evers Douglas F Covey Mariano A Ostuni Jean-Jacques Lacapère Charbel Massaad Michael Schumacher Esther-Marie Steidl Delphine Maux Michel Delaage Christopher E Henderson Rebecca M Pruss

Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disorder characterized by progressive death of cortical and spinal motor neurons, for which there is no effective treatment. Using a cell-based assay for compounds capable of preventing motor neuron cell death in vitro, a collection of approximately 40,000 low-molecular-weight compounds was screened to identify potential small-mol...

Journal: :Annual review of neuroscience 2004
Lucie I Bruijn Timothy M Miller Don W Cleveland

Although Charcot described amyotrophic lateral sclerosis (ALS) more than 130 years ago, the mechanism underlying the characteristic selective degeneration and death of motor neurons in this common adult motor neuron disease has remained a mystery. There is no effective remedy for this progressive, fatal disorder. Modern genetics has now identified mutations in one gene [Cu/Zn superoxide dismuta...

Journal: :Arquivos De Neuro-psiquiatria 2023

Background: Spinal muscular atrophy (SMA) is a genetic motor neuron disease caused by mutations in the SMN1 (Survival Motor Neuron) gene, which leads to hypotonia and muscle weakness with high mortality related respiratory involvement. Gene therapy (GT) (onasemnogeno aberpavovec) for SMA, through an adeno-associated viral vector 9 (AAV9) was recently approved our country, but its safety efficac...

Journal: :iranian rehabilitation journal 0
sahel hemmati tehran . evin . kodakyarblv.social welfare university nasrin amiri tehran - evin blv koodakyar -social welfare university basic science departmant farin solemani tehran - evin blv koodakyar -social welfare university basic science departmant asghar dadkhah pediatric neurorehabilitation research center, university of social welfare and rehabilitation sciences, tehran, iran.

objectives: developmental coordination disorder (dcd) is a motor skill disorder which impacts upon a child, s ability to perform age-appropriate activity of daily living and academic performance. they have problems in gross & fine motors, their upper limb coordination are impaired, too. in this way, we decided to compare motors skills with botmp test in children with dcd and their normal peers....

Journal: :Nihon Naika Gakkai Zasshi 2018

یزدان پناه , پرویز,

The familial spastic paraplegia (FSP) is a heterogeneous group of motor neuron disorders characterized by slow progressive weakness and spasticity of lower limbs. The disorder can appear at any age, but it usually occurs in childhood or early adult life. The genetic pattern of this disease is mainly autosomal dominant trait, but occasionally as an autosomal recessive trait, and very rarely as...

2014
Caroline May Eckhard Nordhoff Swaantje Casjens Michael Turewicz Martin Eisenacher Ralf Gold Thomas Brüning Beate Pesch Christian Stephan Dirk Woitalla Botond Penke Tamás Janáky Dezső Virók László Siklós Jozsef I. Engelhardt Helmut E. Meyer

Amyotrophic lateral sclerosis (ALS), the most common adult-onset motor neuron disorder, is characterized by the progressive and selective loss of upper and lower motor neurons. Diagnosis of this disorder is based on clinical assessment, and the average survival time is less than 3 years. Injections of IgG from ALS patients into mice are known to specifically mark motor neurons. Moreover, IgG ha...

2003
Jau-Shin Lou

Amyotrophic lateral sclerosis (ALS) is a rapidly progressive disease with an average surviving time of 3 to 5 years. The hallmarks of the pathological changes are loss of lower motor neurons in the spinal cord and upper motor neurons(UMN) in the motor cortex. However, structural MRI studies of the spinal cord and the brain often fail to show any changes. Due to lack of objective and quantitativ...

Journal: :Neuron 2011
Priyanka Sabharwal Changhee Lee Sungjin Park Meenakshi Rao Shanthini Sockanathan

The specification of spinal interneuron and motor neuron identities initiates within progenitor cells, while motor neuron subtype diversification is regulated by hierarchical transcriptional programs implemented postmitotically. Here we find that mice lacking GDE2, a six-transmembrane protein that triggers motor neuron generation, exhibit selective losses of distinct motor neuron subtypes, spec...

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