001     157830
005     20230915094042.0
024 7 _ |a 10.1016/bs.pmbts.2020.03.009
|2 doi
024 7 _ |a 10.1016/bs.pmbts.2020.03.009
|2 doi
024 7 _ |a pmid:32620242
|2 pmid
024 7 _ |a 0079-6603
|2 ISSN
024 7 _ |a 1877-1173
|2 ISSN
024 7 _ |a 1878-0814
|2 ISSN
024 7 _ |a 2211-9108
|2 ISSN
037 _ _ |a DZNE-2021-01287
082 _ _ |a 530
100 1 _ |a Rodriguez-Muela, Natalia
|0 P:(DE-2719)9000726
|b 0
|e First author
|u dzne
245 _ _ |a Autophagy in motor neuron diseases
260 _ _ |a Amsterdam ˜[u.a.]œ
|c 2020
|b Elsevier
336 7 _ |a article
|2 DRIVER
336 7 _ |a Output Types/Journal article
|2 DataCite
336 7 _ |a Journal Article
|b journal
|m journal
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|s 1632320839_31140
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|x Review Article
336 7 _ |a ARTICLE
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336 7 _ |a JOURNAL_ARTICLE
|2 ORCID
336 7 _ |a Journal Article
|0 0
|2 EndNote
520 _ _ |a 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.
536 _ _ |a 352 - Disease Mechanisms (POF4-352)
|0 G:(DE-HGF)POF4-352
|c POF4-352
|f POF IV
|x 0
650 _ 2 |a Adult
|2 MeSH
650 _ 2 |a Amyotrophic Lateral Sclerosis: genetics
|2 MeSH
650 _ 2 |a Amyotrophic Lateral Sclerosis: pathology
|2 MeSH
650 _ 2 |a Animals
|2 MeSH
650 _ 2 |a Autophagy: drug effects
|2 MeSH
650 _ 2 |a Autophagy: physiology
|2 MeSH
650 _ 2 |a Autophagy-Related Proteins: genetics
|2 MeSH
650 _ 2 |a Autophagy-Related Proteins: physiology
|2 MeSH
650 _ 2 |a C9orf72 Protein: deficiency
|2 MeSH
650 _ 2 |a C9orf72 Protein: genetics
|2 MeSH
650 _ 2 |a C9orf72 Protein: physiology
|2 MeSH
650 _ 2 |a DNA Repeat Expansion
|2 MeSH
650 _ 2 |a Disease Models, Animal
|2 MeSH
650 _ 2 |a Endocytosis
|2 MeSH
650 _ 2 |a Humans
|2 MeSH
650 _ 2 |a Mice, Transgenic
|2 MeSH
650 _ 2 |a Motor Neuron Disease: genetics
|2 MeSH
650 _ 2 |a Motor Neuron Disease: pathology
|2 MeSH
650 _ 2 |a Muscular Atrophy, Spinal: genetics
|2 MeSH
650 _ 2 |a Muscular Atrophy, Spinal: pathology
|2 MeSH
650 _ 2 |a Mutation
|2 MeSH
650 _ 2 |a Neurodegenerative Diseases: genetics
|2 MeSH
650 _ 2 |a Neurodegenerative Diseases: pathology
|2 MeSH
650 _ 2 |a Organelles
|2 MeSH
650 _ 2 |a RNA-Binding Protein FUS: deficiency
|2 MeSH
650 _ 2 |a RNA-Binding Protein FUS: genetics
|2 MeSH
650 _ 2 |a RNA-Binding Protein FUS: physiology
|2 MeSH
650 _ 2 |a TDP-43 Proteinopathies: genetics
|2 MeSH
650 _ 2 |a TDP-43 Proteinopathies: pathology
|2 MeSH
773 _ _ |a 10.1016/bs.pmbts.2020.03.009
|0 PERI:(DE-600)2474898-5
|p 157-202
|t Progress in molecular biology and translational science
|v 172
|y 2020
|x 1877-1173
909 C O |o oai:pub.dzne.de:157830
|p VDB
910 1 _ |a Deutsches Zentrum für Neurodegenerative Erkrankungen
|0 I:(DE-588)1065079516
|k DZNE
|b 0
|6 P:(DE-2719)9000726
913 1 _ |a DE-HGF
|b Gesundheit
|l Neurodegenerative Diseases
|1 G:(DE-HGF)POF4-350
|0 G:(DE-HGF)POF4-352
|3 G:(DE-HGF)POF4
|2 G:(DE-HGF)POF4-300
|4 G:(DE-HGF)POF
|v Disease Mechanisms
|x 0
914 1 _ |y 2020
915 _ _ |a DBCoverage
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|d 2021-02-02
915 _ _ |a WoS
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915 _ _ |a JCR
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915 _ _ |a DBCoverage
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915 _ _ |a DBCoverage
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915 _ _ |a IF < 5
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920 1 _ |0 I:(DE-2719)1713001
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|l Selective Neuronal Vulnerability in Neurodegenerative Diseases
|x 0
980 _ _ |a journal
980 _ _ |a VDB
980 _ _ |a I:(DE-2719)1713001
980 _ _ |a UNRESTRICTED


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