Amyotrophic lateral sclerosis (ALS), spinal bulbar muscular atrophy (or Kennedy's disease), spinal muscular atrophy and spinal muscular atrophy with respiratory distress 1 are neurodegenerative disorders mainly affecting motor neurons and which currently lack effective therapies. Recent studies in animal models as well as primary and embryonic stem cell models of ALS, utilizing over-expression ...

Background: Adult stem cells (ASC) are undifferentiated cells found throughout the body. These cells are promising tools for cell replacement therapy in neurodegenerative disease. Adipose tissue is the most abundant and accessible source of ASC. This study was conducted to evaluate effect of selegiline on differentiation of adipose-derived stem cells (ADSC) into functional neuron-like cells (NL...

We used anatomical and physiological approaches to characterize nicotinic receptors (AChRs) on Renshaw cells of the neonatal rat spinal cord. Confocal imaging of Renshaw cells, identified by their characteristic pattern of gephyrin immunoreactivity, revealed that these neurons are immuno-positive for the alpha4 and beta2 AChR subunits but not for the alpha7 subunit. We used whole cell recording...

Amyotrophic lateral sclerosis (ALS) is a multifactorial neurodegenerative disease characterized by progressive depletion of motor neurons (MNs). Recent evidence suggests role in ALS pathology for the C-X-C motif chemokine receptor 2 (CXCR2), whose expression was found increased at both mRNA and protein level cortical sporadic patients. Previous findings also showed that inhibition able to preve...

The differentiation of floor plate cells and motor neurons can be induced by Sonic hedgehog (SHH), a secreted signaling protein that undergoes autoproteolytic cleavage to generate amino- and carboxy-terminal products. We have found that both floor plate cells and motor neurons are induced by the amino-terminal cleavage product of SHH (SHH-N). The threshold concentration of SHH-N required for mo...

A new drop-by-drop method was developed to prepare a thermosensitive, chitosan-based hydrogel system that allowed easy encapsulation of NSC-34 cells, supporting their viability and favouring differentiation.

Amyotrophic lateral sclerosis, commonly known as Lou Gehrig's disease, or simply ALS, is a neurodegenerative disease that slowly kills its victims within a few years after diagnosis. This is a complex disease in which every motor neuron in the body is killed over a relatively short time period within 3 years or so, thus killing the victim. This disease can be classified into three main categori...

Respiratory failure is rarely a presenting symptom of motor neuron disease. Seven patients with motor neuron disease who presented with acute respiratory failure of unknown cause and required mechanical ventilation were studied. They all had symptoms and signs suggestive of diaphragmatic weakness. Respiratory involvement seemed disproportionately severe, as six were ambulatory and only three no...

Objective(s): Nowadays much effort is being invested in order to diagnose the mechanisms involved in neural differentiation. By clarifying this, making desired neural cells in vitro and applying them into diverse neurological disorders suffered from neural cell malfunctions could be a feasible choice. Thus, the present study assessed the capability of fetal brain extract (FBE) to induce rat bon...

Differentiation of human pluripotent stem cells (hPSCs) in vitro offers a way to study cell types that are not accessible in living patients. Previous research suggests that hPSCs generate ATP through anaerobic glycolysis, in contrast to mitochondrial oxidative phosphorylation (OXPHOS) in somatic cells; however, specialized cell types have not been assessed. To test if mitobiogenesis is increas...