Spinal muscular atrophy (SMA), a genetic neurodegenerative disorder, is caused by mutations or deletions in the survival of motor neuron 1 (SMN1) gene that result in SMN deficiency. SMN deficiency impairs microtubule networks in Smn-deficient cells and in SMA-like motor neuron cultures. Microtubule defects can be restored by knockdown of the stathmin gene (Stmn), which is upregulated in SMA. Ho...

introduction cholinergic system is one of the important systems of mammalian cns. cholinergic neurons distributed in brain and spinal cord and contributed to principal functions like: consciousness, learning and memory, and motor control. in this study we investigated the differentiation potentiality of mouse embryonic stem cells toward cholinergic neurons. the aim of this study was to evaluate...

Direct differentiation of embryonic stem (ES) cells into functional motor neurons represents a promising resource to study disease mechanisms, to screen new drug compounds, and to develop new therapies for motor neuron diseases such as spinal muscular atrophy (SMA) and amyotrophic lateral sclerosis (ALS). Many current protocols use a combination of retinoic acid (RA) and sonic hedgehog (Shh) to...

Cell replacement using stem cells is a promising therapeutic approach to treat degenerative motor neuron (MN) disorders, such as amyotrophic lateral sclerosis and spinal cord injury. Human bone marrow-derived mesenchymal stem cells (hMSCs) are a desirable cell source for autologous cell replacement therapy to treat nervous system injury due to their plasticity, low immunogenicity, and a lower r...

Different somatic motor neuron subpopulations show a differential vulnerability to degeneration in diseases such as amyotrophic lateral sclerosis, spinal muscular atrophy and spinobulbar muscular atrophy. Studies in mutant superoxide dismutase 1 over-expressing amyotrophic lateral sclerosis model mice indicate that initiation of disease is intrinsic to motor neurons, while progression is promot...

The topographic assembly of neural circuits is dependent upon the generation of specific neuronal subtypes, each subtype displaying unique properties that direct the formation of selective connections with appropriate target cells. Studies of motor neuron development in the spinal cord have begun to elucidate the molecular mechanisms involved in controlling motor projections. In this review, we...

background: bone marrow stromal stem cells (bmsc) are appropriate source of multipotent stem cells that are ideally suited for use in various cell-based therapies. it can be differentiated into neuronal-like cells under appropriate conditions. this study examined the effectiveness of co-stimulation of creatine and retinoic acid in increasing the differentiation of bmsc into gabaergic neuron-lik...

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...

During spinal cord development, ventral neural progenitor cells that express the transcription factors Olig1 and Olig2, called pMN progenitors, produce motor neurons and then oligodendrocytes. Whether motor neurons and oligodendrocytes arise from common or distinct progenitors in vivo is not known. Using zebrafish, we found that motor neurons and oligodendrocytes are produced sequentially by di...