in this study organotypic adult spinal cord slices were used to investigate whether caspases could participate in the apoptosis of motor neurons. the thoracic region of spinal cord was sliced using a tissue chopper and cultured in a medium for 6h. morphological and biochemical features of apoptosis were assessed by fluorescent staining and terminal deoxynucleotidyl nick end labeling (tunel) met...

Objective(s): Cell therapy has provided clinical applications to the treatment of motor neuron diseases. The current obstacle in stem cell therapy is to direct differentiation of stem cells into neurons in the neurodegenerative disorders. Biomaterial scaffolds can improve cell differentiation and are widely used in translational medicine and tissue engineering. The aim...

Spinal muscular atrophy (SMA) is a genetic disorder which affect nervous system and is characterized with progressive distal motor neuron weakness. The survival motor neuron (SMN) protein level reduces in patients with SMA. Two different genes code survival motor neuron protein in human genome. Skeletal and intercostal muscles denervation lead to weakness, hypotony, hyporeflexia, respiratory fa...

spinal muscular atrophy (sma) is a genetic disorder which affect nervous system and is characterized with progressive distal motor neuron weakness. the survival motor neuron (smn) protein level reduces in patients with sma. two different genes code survival motor neuron protein in human genome. skeletal and intercostal muscles denervation lead to weakness, hypotony, hyporeflexia, respiratory fa...

objective(s) motor deficit and neuron degeneration is seen after nerve transection. the aim of this study is to determine whether a poled polyvinelidene fluoride (pvdf) tube with other supportive strategies can protect the neuronal morphology and motor function after sciatic nerve transaction in rats. materials and methods after transection of the left sciatic nerve in 60 male wistar rats (200-...

background: sandhoff disease is an autosomal recessive disorder caused by β-hexosaminidase deficiency and accumulation of gm2 ganglioside resulting in progressive motor neuron manifestations and death from respiratory failure and infections in infantiles. pathogenic mutations in hexb gene were observed which leads to enzyme activity reduction and interruption of normal metabolic cycle of gm2 ga...

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 cultur...

amyotrophic lateral sclerosis (als), the most common form of motor neuron disease, is a progressive and devastating disease involving both lower and upper motor neurons, typically following a relentless progression towards death. therefore, all efforts must be made by the clinician to exclude alternative and more treatable entities. als with laboratory abnormalities of uncertain significance is...

objective we describe three patients with very severe spinal muscular atrophy (sma) presented with reduced fetal movement in utero, profound hypotonia, severe weakness and respiratory insufficiency at birth. in all infants, electrodiagnostic studies were compatible with a neurogenic pattern. in genetic studies, all cases had homozygous deletions of exons 7 and 8 of survival motor neuron (smn) a...

amyotrophic lateral sclerosis (als) misdiagnosis has many broad implications for the patient and the neurologist. potentially curative treatments exist for certain als mimic syndromes, but delay in starting these therapies may have an unfavorable effect on outcome. hence, it is important to exclude similar conditions. in this review, we discuss some of the important mimics of als.