LEADER 04305nam 2200577 450 001 9910137237503321 005 20230621135410.0 010 $a9782889193769 (ebook) 035 $a(CKB)3710000000506266 035 $a(SSID)ssj0001666248 035 $a(PQKBManifestationID)16455026 035 $a(PQKBTitleCode)TC0001666248 035 $a(PQKBWorkID)14999949 035 $a(PQKB)10067355 035 $a(WaSeSS)IndRDA00056293 035 $a(oapen)https://directory.doabooks.org/handle/20.500.12854/42864 035 $a(EXLCZ)993710000000506266 100 $a20160829d2015 uy | 101 0 $aeng 135 $aur||||||||||| 181 $ctxt 182 $cc 183 $acr 200 10$aCellular and molecular mechanisms of motor neuron death in amyotrophic lateral sclerosis$b[electronic resource] /$fRicardo Tapia 210 $cFrontiers Media SA$d2015 210 31$aFrance :$cFrontiers Media SA,$d2015 215 $a1 online resource (190 pages) $cillustrations 225 0 $aFrontiers Research Topics,$x1664-8714 300 $aBibliographic Level Mode of Issuance: Monograph 320 $aIncludes bibliographical references. 330 $aAmyotrophic lateral sclerosis (ALS), which was described since 1869 by Jean Martin Charcot, is a devastating neurodegenerative disease characterized by the selective and progressive loss of upper and lower motor neurons of the cerebral cortex, brainstem and the spinal cord. The cognitive process is not affected and is not merely the result of aging because may occur at young ages. The only known cause of the disease is associated with genetic mutations, mainly in the gene encoding superoxide dismutase 1 (familial ALS), whereas there is no known cause of the sporadic form of ALS (SALS), which comprises >90% of cases. Both ALS types develop similar histopathological and clinical characteristics, and there is no treatment or prevention of the disease. Because effective treatments for ALS, as for other neurodegenerative diseases, can only result from the knowledge of their cellular and molecular pathophysiological mechanisms, research on such mechanisms is essential. Although progress in neurochemical, physiological and clinical investigations in the last decades has identified several mechanisms that seem to be involved in the cell death process, such as glutamate-mediated excitotoxicity, alterations of inhibitory circuits, inflammatory events, axonal transport deficits, oxidative stress, mitochondrial dysfunction and energy failure, the understanding of the origin and temporal progress of the disease is still incomplete and insufficient. Clearly, there is a need of further experimental models and approaches to discern the importance of such mechanisms and to discover the factors that determine the selective death of motor neurons characteristic of ALS, in contrast to other neurodegenerative diseases such as Parkinson?s and Alzheimer?s disease. Whereas studies in vitro in cell cultures, tissue slices or organotypic preparations can give useful information regarding cellular and molecular mechanisms, the experiments in living animal models obviously reflect more closely the situation in the human disease, provided that the symptoms and their development during time mimics as close as possible those of the human disease. It is necessary to correlate the experimental findings in vitro with those in vivo, as well as those obtained in genetic models with those in non-genetic models, aiming at designing and testing therapeutic strategies based on the results obtained. 606 $aNeurology$2HILCC 606 $aMedicine$2HILCC 606 $aHealth & Biological Sciences$2HILCC 610 $atrophic factors 610 $amotor neuron degeneration 610 $askeletal 610 $aneuroinflammation 610 $amuscle 610 $agenetic expression 610 $aspinal cord 610 $aamyotrophic lateral sclerosis (ALS) 615 7$aNeurology 615 7$aMedicine 615 7$aHealth & Biological Sciences 700 $aTapia$b Ricardo$0941107 801 0$bPQKB 801 2$bUkMaJRU 912 $a9910137237503321 996 $aCellular and molecular mechanisms of motor neuron death in amyotrophic lateral sclerosis$92122519 997 $aUNINA