Immunological Aspects in Amyotrophic Lateral Sclerosis
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REVIEW ARTICLE
Immunological Aspects in Amyotrophic Lateral Sclerosis Maria Carolina O. Rodrigues & Júlio C. Voltarelli & Paul R. Sanberg & Cesario V. Borlongan & Svitlana Garbuzova-Davis
Received: 15 March 2012 / Revised: 7 April 2012 / Accepted: 11 April 2012 / Published online: 3 May 2012 # Springer Science+Business Media, LLC 2012
Abstract Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by progressive motor neuron death, leading to muscle atrophy, paralysis, and death usually within 3 to 5 years after diagnosis. Most cases are sporadic, with still undefined etiopathogenesis. Both the innate and adaptive immune systems are involved in ALS, with special participation of T lymphocytes and microglia. Inflammation plays a dual role in the disease, protective and T regulatory cell rich in the early stages and deleterious as disease progresses. Attempts to modulate immune/inflammatory system response are reported in the literature, and while beneficial effects are achieved in ALS animal models, M. C. O. Rodrigues : J. C. Voltarelli Department of Internal Medicine, Ribeirão Preto School of Medicine, University of São Paulo, São Paulo, Brazil P. R. Sanberg : C. V. Borlongan : S. Garbuzova-Davis (*) Center of Excellence for Aging and Brain Repair, Department of Neurosurgery and Brain Repair, Morsani College of Medicine, University of South Florida, 12901 Bruce B. Downs Blvd., Tampa, FL 33612, USA e-mail: [email protected] S. Garbuzova-Davis Department of Molecular Pharmacology and Physiology, Morsani College of Medicine, University of South Florida, Tampa, FL 33612, USA P. R. Sanberg Department of Pathology and Cell Biology, Morsani College of Medicine, University of South Florida, Tampa, FL 33612, USA P. R. Sanberg Department of Psychiatry, Morsani College of Medicine, University of South Florida, Tampa, FL 33612, USA
results of most clinical trials have been disappointing. The impaired blood–brain barrier is an important feature in the pathogenesis of ALS and likely affects the immune system response. The present review describes the role of the immune system in ALS pathogenesis and the tight coupling of immunity and central nervous system barrier function. Keywords Amyotrophic lateral sclerosis (ALS) . Immune system . Inflammation . Lymphocytes . Microglia . Blood– brain barrier (BBB)
Introduction Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by progressive motor neuron death, leading to muscle atrophy, paralysis, and death usually within 3 to 5 years after diagnosis. It affects approximately 2 per 100,000 people [1–5] and is slightly more common in men than in women [6]. Only about 5 to 10 % of the cases are genetically linked, 20 % of which show missense mutations in the Cu/Zn superoxide dismutase (SOD1) gene on chromosome 21 [7]. Sporadic cases predominate, with still undefined etiology. Numerous pathological mechanisms have been described in ALS [8–16]; however, their exact contributions to motor neuron degeneration and the events trigger
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