Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0026850 (muscular dystrophy)
 5,870 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Neuromuscular diseases are a broadly defined group of disorders that all involve injury or dysfunction of peripheral nerves or muscle. The site of injury can be in the cell bodies (i.e., amyotrophic lateral sclerosis [ALS] or sensory ganglionopathies), axons (i.e., axonal peripheral neuropathies or brachial plexopathies), Schwann cells (i.e., chronic inflammatory demyelinating polyradiculoneuropathy), neuromuscular junction (i.e., myasthenia gravis or Lambert-Eaton myasthenic syndrome), muscle (i.e., inflammatory myopathy or muscular dystrophy), or any combination of these sites. Some neuromuscular diseases are also associated with central nervous system disease, such as ALS, but most are restricted to the peripheral nervous system. The multitude of possible sites of injury can make neuromuscular diseases difficult to diagnose. Here the author reviews key features of the clinical presentation that help localize the site of injury and some basic tenets of electromyography. He then shares several pearls in diagnosing and treating patients with specific neuromuscular diseases.
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PMID:Neuromuscular Diseases. 2770 95

The control of polarized human neurite/axon development at the single neuron level is critical in geographically directing signal propagation in engineered neural networks, for both in vitro and in vivo applications. While there is an increasing need to exert control over axonal growth for the successful development and establishment of integrative and functional in vitro systems, controlled, polarized distribution of either human-derived neurons or motoneurons in vitro has yet to be reported. In this study, we established the polarized distribution of stem cell derived human motoneurons, using a patterned surface, and maintained the cells in a serum-free system. A surface pattern with defined polarity was developed using self-assembled monolayers (SAMs). A cell permissive SAM, DETA (trimethoxysilyl propyldiethylenetri-amine), combined with photolithography and a nonpermissive fluorinated silane, 13F (tridecafluoro-1,1,2,2-tetrahydroctyl-1-dimethylchloro-silane), generated a surface where neurons only adhered to the designed attachment sites and did so with preferred orientation. In addition, 75% of the cells attached to the patterns were motoneurons compared to their percentage in the standard unpatterned surface which was used as a control condition (20%), demonstrating the preference of these human motoneurons in adhering to the patterns. The ability to dictate the distribution and polarity of human motoneurons will be essential to the engineering of human-based functional in vitro systems in which the control of signal propagation is necessary but more importantly for cell implantation studies. Such systems will greatly benefit the study of motor function as well as aid the development of high-throughput systems for drug screening and test beds for use in preclinical studies related to conditions such as spinal cord injury, ALS, and muscular dystrophy.
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PMID:Polarity Induced in Human Stem Cell Derived Motoneurons on Patterned Self-Assembled Monolayers. 3106 82


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