title: [Amyotrophic lateral sclerosis: is the astrocyte the cell primarily involved?].
abstract: So far, amyotrophic lateral sclerosis (ALS) is thought as due to a primary insult of the motor neurons. None of its pathogenic processes proved to be the cause of the illness, nor can be blamed environmental agents. Motor neurons die by apoptosis, leaving the possibility that their death might be due to an unfriendly environment, unable to sustain their health, rather than being directly targeted themselves. These reasons justify an examination of the astrocytes, because they have the most important role controlling the neurons' environment. It is known that astrocytes are plastic, enslaving their functions to the requirements of the neurons to which they are related. Each population of astrocytes is unique, and if it were affected the consequences would reach the neurons that it normally sustains. In regard to the motor neurons, this situation would lead to a disturbed production and release of astrocytic neurotransmitters and transporters, impairing nutritional and trophic support as well. For explaining the spreading of muscle symptoms in ALS, correlated with the type of spreading observed at the cortical and spinal motor neurons pools, the present hypotheses suggests that the illness-causing process is spreading among astrocytes, through their gap junctions, depriving the motor neurons of their support. Also it is postulated that a normal astrocytic protein becomes misfolded and infectious, inducing the misfolding of its wild type, travelling from one protoplasmatic astrocyte to another and to the fibrous astrocytes encircling the pyramidal pathway which joints the upper and lower motoneurones.
authors.affiliation: Sector Neurología, Instituto de Investigaciones Cardiológicas Alberto Taquini (ININCA, UBA-CONICET), Facultad de Medicina, Universidad de Buenos Aires. E-mail: rsica@fmed.uba.ar.
authors.forename: Roberto E
authors.initials: RE
authors.lastname: Sica
mesh_terms: D000690:Amyotrophic Lateral Sclerosis / Q000473:pathology*; D001253:Astrocytes / Q000473:pathology* / Q000502:physiology; D060833:Cellular Microenvironment; D006801:Humans; D008954:Models, Biological; D009046:Motor Neurons / Q000473:pathology / Q000502:physiology
keywords: amyotrophic lateral sclerosis; astrocyte; pathogenesis