The p75 neurotrophin receptor (p75(NTR); also known as NGFR) can mediate neuronal apoptosis in disease or following trauma, and facilitate survival through interactions with Trk receptors. Here we tested the ability of a p75(NTR)-derived trophic cell-permeable peptide, c29, to inhibit p75(NTR)-mediated motor neuron death. Acute c29 application to axotomized motor neuron axons decreased cell dea...

introduction: the aim of this study was evaluate the ability of notochord to induce neural induction and/or differentiation of mouse embryonic stem cell to neuron and motor neuron, respectively. methods: in order to produce embryoid bodies, es cells line royan b1 were grown in suspension in the absence of lif for 4 days. ebs were divided into 4 groups. ebs in group 1 & 2 were further cultured i...

OBJECTIVE To describe the clinical and electrophysiologic features of patients with inclusion body myositis that was misinterpreted as motor neuron disease. PATIENTS AND METHODS We retrospectively retrieved the medical records of 70 patients with a pathologic diagnosis of inclusion body myositis. From this group, we selected those who had been first diagnosed as having motor neuron disease or...

Motor neuron disease causes widespread degeneration of motor neurons within both the brain and spinal cord. Brain and spinal cord MRI were performed in 11 patients with motor neuron disease, and in 17 controls. Symmetric areas of high signal within the corticospinal tracts were found in nine patients on T2 weighted spin echo (SE) or fast spin echo (FSE) images of the brain and in eight on T2 or...

Introduction: Profilin1 (PFN1) is a ubiquitously expressed protein known for its function as a regulator of actin polymerization and dynamics. A recent discovery linked mutant PFN1 to Amyotrophic Lateral Sclerosis (ALS), which is a fatal and progressive motor neuron disease. We have also demonstrated that Gly118Val mutation in PFN1 is a cause of ALS, and the formation of aggregates containing m...

Kennedy's disease is an X-linked, neurodegenerative disorder, characterised by lower motor neuron syndrome. This report gives the clinical details of six male patients with Kennedy's disease diagnosed at Princess Margaret Hospital. Three were initially diagnosed with other neurological diseases, with the diagnosis of Kennedy's disease made after genetic testing. This hereditary disease should b...

The mechanism of selective and age-dependent motor neuron degeneration in human amyotrophic lateral sclerosis (ALS) has not been defined and the role of glutathione (GSH) in association with motor neuron death remains largely unknown. A motor neuron-like cell culture system and a transgenic mouse model were used to study the effect of cellular GSH alteration on motor neuron cell death. Exposure...

Movement provides the only means we have to interact with both the world and other people. Such interactions can be hard-wired or learned through experience with the environment. Learning allows us to adapt to a changing physical environment as well as to novel conventions developed by society. Here we review motor learning from a computational perspective, exploring the need for motor learning...

AIM Presentation of a prenatally diagnosed case of Werdnig-Hoffmann disease, the most severe type of spinal muscular atrophy. METHODS DNA obtained from cultivated amniocytes was analyzed for deletions in the survival motor neuron gene and neuronal apoptosis inhibitory protein gene. RESULTS The fetus was diagnosed as an affected homozygote for deletions in exon 7 and exon 8 of the survival m...