title: Why do motor neurons degenerate? Actualization in the pathogenesis of amyotrophic lateral sclerosis.
abstract: BACKGROUND
  Amyotrophic lateral sclerosis (ALS) is the most common neurodegenerative disease affecting motor neurons. Although a small proportion of ALS cases are familial in origin and linked to mutations in specific genes, most cases are sporadic and have a multifactorial aetiology. Some recent studies have increased our knowledge of ALS pathogenesis and raised the question of whether this disorder is a proteinopathy, a ribonucleopathy, an axonopathy, or a disease related to the neuronal microenvironment.
  
  
  METHODS
  This article presents a review of ALS pathogenesis. To this end, we have reviewed published articles describing either ALS patients or ALS animal models and we discuss how the main cellular pathways (gene processing, protein metabolism, oxidative stress, axonal transport, relationship with neuronal microenvironment) may be involved in motor neurons degeneration.
  
  
  CONCLUSIONS
  ALS pathogenesis has not been fully elucidated. Recent studies suggest that although initial triggers may differ among patients, the final motor neurons degeneration mechanisms are similar in most patients once the disease is fully established.
authors: Servicio de Neurología, Hospital Universitario Marqués de Valdecilla, Instituto de Investigación Valdecilla (IDIVAL), Universidad de Cantabria, Santander, España; Centro de Investigación Biomédica en Red sobre Enfermedades Neurodegenerativas (CIBERNED), España. Electronic address: javier.riancho86@gmail.com.
authors: J
authors: J
authors: Riancho
authors: Departamento de Anatomía y Biología Celular, Universidad de Cantabria , Santander, España.
authors: I
authors: I
authors: Gonzalo
authors: Departamento de Anatomía y Biología Celular, Universidad de Cantabria , Santander, España; Centro de Investigación Biomédica en Red sobre Enfermedades Neurodegenerativas (CIBERNED), España.
authors: M
authors: M
authors: Ruiz-Soto
authors: Servicio de Neurología, Hospital Universitario Marqués de Valdecilla, Instituto de Investigación Valdecilla (IDIVAL), Universidad de Cantabria, Santander, España; Centro de Investigación Biomédica en Red sobre Enfermedades Neurodegenerativas (CIBERNED), España.
authors: J
authors: J
authors: Berciano
mesh_terms: D000690:Amyotrophic Lateral Sclerosis / Q000235:genetics / Q000378:metabolism / Q000473:pathology*; D000818:Animals; D004195:Disease Models, Animal; D006801:Humans; D009046:Motor Neurons / Q000473:pathology*
keywords: Amyotrophic lateral sclerosis; Esclerosis lateral amiotrófica; Motoneurona; Motor neuron; Neurodegeneración; Neurodegeneration