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ALS (Amyotrophic Lateral Sclerosis)

LouGehrig's disease ALS MUSCLE Amyotrophic Lateral Sclerosis (ALS) or Lou Gehrig's disease is a progressive neurodegenerative disorder. 30,000 Americans NOW have ALS The earliest known description of ALS was in the 1800s. Common symptoms of ALS are muscle weakness, stiffness, and eventual paralysis. Motor neurons extend from the brain to the spinal cord and release neurotransmitters initiating muscle contraction. In ALS these motor neurons gradually degenerate and die hindering the brain's control of muscle movements. No known cause for ALS • ALS is most commonly a sporadic disease. • Only 5-10% of ALS cases are inherited (familial ALS). • Incidence: 2 in 100,000 people develop ALS each year. || ||| Although there is still no precise known cause, mutation in gene SOD1 is believed to be the major cause of ALS. Following SOD1, mutations in the genes FUS and TARDBP 68% 11% are common causes of familial ALS. Of the genes discovered, 68% Of the genes discovered, 11% contribute to familial ALS contribute to sporadic ALS TIMELINE OF GENE DISCOVERIES IMPLICATED IN ALS 1993 2002 2008 2010 2011 2014 De novo TARDBP VCP C9ORF72 PFN1 SOD1 SOD1 & FUS Molecular mechanisms of motor neuron injury in ALS Genetic factors Oxidative stress Mitochondrial dysfunction Excitotoxicity Protein aggregation Dysregulated endosomal trafficking Impaired axonal transport Neuroinflammation Endoplasmic reticulum stress Dysregulated transcription and RNA processing No known cure for ALS However, in late 1995 Riluzone III||||| became the first FDA 0000 -approved drug to treat ALS. This drug was shown to slow down the progression of ALS, prolonging patient life by at least a few months. References - Renton AE, Chiò A, Traynor BJ. State of play in amyotrophic lateral sclerosis genetics. Nat Neurosci. 2014 -Vucic S, Rothstein JD, Kiernan MC. Advances in treating amyotrophic lateral sclerosis: insights from pathophysiological studies. Trends Neurosci. 2014 - Ferraiuolo L, Kirby J, Grierson AJ, Sendtner M, Shaw PJ. Molecular pathways of motor neuron injury in amyotrophic lateral sclerosis. Nat Rev Neurol. 2011 -Andersen PM, Al-Chalabi A. Clinical genetics of amyotrophic lateral sclerosis: what do we really know? Nat Rev Neurol. 2011 -Dion PA, Daoud H, Rouleau GA. Genetics of motor neuron disorders: new insights into pathogenic mechanisms. Nat Rev Genet. 2009 © 2014

ALS (Amyotrophic Lateral Sclerosis)

shared by J.L on Sep 02
With the ongoing ALS Ice Bucket Challenge, Knowing Neurons team explores the neurological basis of ALS (Amyotrophic Lateral Sclerosis)!


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