Amyotrophic Lateral Sclerosis (ALS) is a motor neuron disease that affects the skeletal muscle nerve cell. In which neurons in the brain and spinal cord that control voluntary movement (LMN and UMN) gradually deteriorate. Impending symptoms like twitching, cramping or stiffness of muscles, muscle weakness, difficulty in chewing or swallowing etc. Approximately 5,600 people are diagnosed with ALS in the US every year. There are 20% more men cases than women with ALS. Principally two recognised forms of ALS are: (A) sporadic (B) familial. The sporadic form accounts for 90% and residual 10% for familial form. It can be diagnosed by electrical and biochemical methods like electromyography, nerve conduction velocity of L/UMN, MRI scan, proteomics and metabolomics which involve potential biomarkers SOD1 gene, ALS2 gene, ALS6 gene. Presently reluzole is the only FDA approved drug for treatment which acts by preventing damage to motor neurons by obstructing glutamate release. Certain drugs under trials are TCH386, topiramate, IGF-1, BDNF-IT, pentoxyfilline etc. Herbal drugs retaining tonic properties help to treat symptoms of ALS like cannabis, licorice, yisui tang etc. Models used to study ALS are neurotoxin, viral, autoimmune, naturally occurring motor neuron disease models and transgenic animal models like SOD1 gene overexpression and human neuro-filament heavy gene expression. Novel targeting therapies aims for glutamate targets, RNA targets, protein misfolding and stem cells therapy. In the present article, an attempt has been made to emphasize on ALS, its animal models and novel targeting site for future drug development.
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