J Riancho1, I Gonzalo2, M Ruiz-Soto3, J Berciano4. 1. 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. 2. Departamento de Anatomía y Biología Celular, Universidad de Cantabria , Santander, España. 3. Departamento de Anatomía y Biología Celular, Universidad de Cantabria , Santander, España; Centro de Investigación Biomédica en Red sobre Enfermedades Neurodegenerativas (CIBERNED), España. 4. 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.
Abstract
INTRODUCTION: 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. DEVELOPMENT: 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.
INTRODUCTION: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. DEVELOPMENT: This article presents a review of ALS pathogenesis. To this end, we have reviewed published articles describing either ALSpatients 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: Erwin A van Vliet; Anand M Iyer; Lucia Mesarosova; Hilal Çolakoglu; Jasper J Anink; Olaf van Tellingen; Nicholas J Maragakis; Jeremy Shefner; Ton Bunt; Eleonora Aronica Journal: J Neuropathol Exp Neurol Date: 2020-03-01 Impact factor: 3.685
Authors: Marco Orsini; Ana Carolina Andorinho de Freitas Ferreira; Osvaldo J M Nascimento; Jano Alves de Souza; Thaís Nascimento Magalhães; Anna Carolina Damm de Assis; Larissa Kozow Westin; Bruno Pessoa; Acary Bulle Oliveira; Rossano Fiorelli; Marcos R G de Freitas; Juliana Bittencourt; Stenio Fiorelli; Maria Fernanda Freitas Ferreira Moreira; Pedro Ribeiro Journal: Neurol Int Date: 2016-06-29