157830 20230915094042.0 10.1016/bs.pmbts.2020.03.009 doi 10.1016/bs.pmbts.2020.03.009 doi pmid:32620242 pmid 0079-6603 ISSN 1877-1173 ISSN 1878-0814 ISSN 2211-9108 ISSN DZNE-2021-01287 530 Rodriguez-Muela, Natalia P:(DE-2719)9000726 0 First author dzne Autophagy in motor neuron diseases Amsterdam ˜[u.a.]œ 2020 Elsevier article DRIVER Output Types/Journal article DataCite Journal Article journal journal PUB:(DE-HGF)16 1632320839_31140 PUB:(DE-HGF) Review Article ARTICLE BibTeX JOURNAL_ARTICLE ORCID Journal Article 0 EndNote Motor neuron diseases (MNDs) are a wide group of neurodegenerative disorders characterized by the degeneration of a specific neuronal type located in the central nervous system, the motor neuron (MN). There are two main types of MNs, spinal and cortical MNs and depending on the type of MND, one or both types are affected. Cortical MNs innervate spinal MNs and these control a variety of cellular targets, being skeletal muscle their main one which is also affected in MNDs. A correct functionality of autophagy is necessary for the survival of all cellular types and it is particularly crucial for neurons, given their postmitotic and highly specialized nature. Numerous studies have identified alterations of autophagy activity in multiple MNDs. The scientific community has been particularly prolific in reporting the role that autophagy plays in the most common adult MND, amyotrophic lateral sclerosis, although many studies have started to identify physiological and pathological functions of this catabolic system in other MNDs, such as spinal muscular atrophy and spinal and bulbar muscular atrophy. The degradation of selective cargo by autophagy and how this process is altered upon the presence of MND-causing mutations is currently also a matter of intense investigation, particularly regarding the selective autophagic clearance of mitochondria. Thorough reviews on this field have been recently published. This chapter will cover the current knowledge on the functionality of autophagy and lysosomal homeostasis in the main MNDs and other autophagy-related topics in the MND field that have risen special interest in the research community. 352 - Disease Mechanisms (POF4-352) G:(DE-HGF)POF4-352 POF4-352 POF IV 0 Adult MeSH Amyotrophic Lateral Sclerosis: genetics MeSH Amyotrophic Lateral Sclerosis: pathology MeSH Animals MeSH Autophagy: drug effects MeSH Autophagy: physiology MeSH Autophagy-Related Proteins: genetics MeSH Autophagy-Related Proteins: physiology MeSH C9orf72 Protein: deficiency MeSH C9orf72 Protein: genetics MeSH C9orf72 Protein: physiology MeSH DNA Repeat Expansion MeSH Disease Models, Animal MeSH Endocytosis MeSH Humans MeSH Mice, Transgenic MeSH Motor Neuron Disease: genetics MeSH Motor Neuron Disease: pathology MeSH Muscular Atrophy, Spinal: genetics MeSH Muscular Atrophy, Spinal: pathology MeSH Mutation MeSH Neurodegenerative Diseases: genetics MeSH Neurodegenerative Diseases: pathology MeSH Organelles MeSH RNA-Binding Protein FUS: deficiency MeSH RNA-Binding Protein FUS: genetics MeSH RNA-Binding Protein FUS: physiology MeSH TDP-43 Proteinopathies: genetics MeSH TDP-43 Proteinopathies: pathology MeSH 10.1016/bs.pmbts.2020.03.009 PERI:(DE-600)2474898-5 157-202 Progress in molecular biology and translational science 172 2020 1877-1173 oai:pub.dzne.de:157830 VDB Deutsches Zentrum für Neurodegenerative Erkrankungen I:(DE-588)1065079516 DZNE 0 P:(DE-2719)9000726 DE-HGF Gesundheit Neurodegenerative Diseases G:(DE-HGF)POF4-350 G:(DE-HGF)POF4-352 G:(DE-HGF)POF4 G:(DE-HGF)POF4-300 G:(DE-HGF)POF Disease Mechanisms 0 2020 DBCoverage StatID:(DE-HGF)0160 StatID Essential Science Indicators 2021-02-02 WoS StatID:(DE-HGF)0113 StatID Science Citation Index Expanded 2021-02-02 JCR StatID:(DE-HGF)0100 StatID PROG MOL BIOL TRANSL : 2021 2023-03-30 DBCoverage StatID:(DE-HGF)0200 StatID SCOPUS 2023-03-30 DBCoverage StatID:(DE-HGF)0300 StatID Medline 2023-03-30 IF < 5 StatID:(DE-HGF)9900 StatID 2023-03-30 I:(DE-2719)1713001 AG Rodriguez-Muela Selective Neuronal Vulnerability in Neurodegenerative Diseases 0 journal VDB I:(DE-2719)1713001 UNRESTRICTED