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000157830 0247_ $$2doi$$a10.1016/bs.pmbts.2020.03.009
000157830 0247_ $$2doi$$a10.1016/bs.pmbts.2020.03.009 
000157830 0247_ $$2pmid$$apmid:32620242
000157830 0247_ $$2ISSN$$a0079-6603
000157830 0247_ $$2ISSN$$a1877-1173
000157830 0247_ $$2ISSN$$a1878-0814
000157830 0247_ $$2ISSN$$a2211-9108
000157830 037__ $$aDZNE-2021-01287
000157830 082__ $$a530
000157830 1001_ $$0P:(DE-2719)9000726$$aRodriguez-Muela, Natalia$$b0$$eFirst author$$udzne
000157830 245__ $$aAutophagy in motor neuron diseases
000157830 260__ $$aAmsterdam ˜[u.a.]œ$$bElsevier$$c2020
000157830 3367_ $$2DRIVER$$aarticle
000157830 3367_ $$2DataCite$$aOutput Types/Journal article
000157830 3367_ $$0PUB:(DE-HGF)16$$2PUB:(DE-HGF)$$aJournal Article$$bjournal$$mjournal$$s1632320839_31140$$xReview Article
000157830 3367_ $$2BibTeX$$aARTICLE
000157830 3367_ $$2ORCID$$aJOURNAL_ARTICLE
000157830 3367_ $$00$$2EndNote$$aJournal Article
000157830 520__ $$aMotor 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.
000157830 536__ $$0G:(DE-HGF)POF4-352$$a352 - Disease Mechanisms (POF4-352)$$cPOF4-352$$fPOF IV$$x0
000157830 650_2 $$2MeSH$$aAdult
000157830 650_2 $$2MeSH$$aAmyotrophic Lateral Sclerosis: genetics
000157830 650_2 $$2MeSH$$aAmyotrophic Lateral Sclerosis: pathology
000157830 650_2 $$2MeSH$$aAnimals
000157830 650_2 $$2MeSH$$aAutophagy: drug effects
000157830 650_2 $$2MeSH$$aAutophagy: physiology
000157830 650_2 $$2MeSH$$aAutophagy-Related Proteins: genetics
000157830 650_2 $$2MeSH$$aAutophagy-Related Proteins: physiology
000157830 650_2 $$2MeSH$$aC9orf72 Protein: deficiency
000157830 650_2 $$2MeSH$$aC9orf72 Protein: genetics
000157830 650_2 $$2MeSH$$aC9orf72 Protein: physiology
000157830 650_2 $$2MeSH$$aDNA Repeat Expansion
000157830 650_2 $$2MeSH$$aDisease Models, Animal
000157830 650_2 $$2MeSH$$aEndocytosis
000157830 650_2 $$2MeSH$$aHumans
000157830 650_2 $$2MeSH$$aMice, Transgenic
000157830 650_2 $$2MeSH$$aMotor Neuron Disease: genetics
000157830 650_2 $$2MeSH$$aMotor Neuron Disease: pathology
000157830 650_2 $$2MeSH$$aMuscular Atrophy, Spinal: genetics
000157830 650_2 $$2MeSH$$aMuscular Atrophy, Spinal: pathology
000157830 650_2 $$2MeSH$$aMutation
000157830 650_2 $$2MeSH$$aNeurodegenerative Diseases: genetics
000157830 650_2 $$2MeSH$$aNeurodegenerative Diseases: pathology
000157830 650_2 $$2MeSH$$aOrganelles
000157830 650_2 $$2MeSH$$aRNA-Binding Protein FUS: deficiency
000157830 650_2 $$2MeSH$$aRNA-Binding Protein FUS: genetics
000157830 650_2 $$2MeSH$$aRNA-Binding Protein FUS: physiology
000157830 650_2 $$2MeSH$$aTDP-43 Proteinopathies: genetics
000157830 650_2 $$2MeSH$$aTDP-43 Proteinopathies: pathology
000157830 773__ $$0PERI:(DE-600)2474898-5$$a10.1016/bs.pmbts.2020.03.009 $$p157-202$$tProgress in molecular biology and translational science$$v172$$x1877-1173$$y2020
000157830 909CO $$ooai:pub.dzne.de:157830$$pVDB
000157830 9101_ $$0I:(DE-588)1065079516$$6P:(DE-2719)9000726$$aDeutsches Zentrum für Neurodegenerative Erkrankungen$$b0$$kDZNE
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000157830 9141_ $$y2020
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000157830 9201_ $$0I:(DE-2719)1713001$$kAG Rodriguez-Muela$$lSelective Neuronal Vulnerability in Neurodegenerative Diseases$$x0
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