000284047 001__ 284047
000284047 005__ 20260122092457.0
000284047 0247_ $$2doi$$a10.1111/febs.70239
000284047 0247_ $$2pmid$$apmid:40891506
000284047 0247_ $$2ISSN$$a0014-2956
000284047 0247_ $$2ISSN$$a0945-5795
000284047 0247_ $$2ISSN$$a1432-1033
000284047 0247_ $$2ISSN$$a1742-464X
000284047 0247_ $$2ISSN$$a1742-4658
000284047 037__ $$aDZNE-2026-00082
000284047 041__ $$aEnglish
000284047 082__ $$a610
000284047 1001_ $$0P:(DE-2719)2811957$$aPir, Ghulam Jeelani$$b0
000284047 245__ $$aTDP-43 proteinopathies and neurodegeneration: insights from Caenorhabditis elegans models.
000284047 260__ $$aOxford [u.a.]$$bWiley-Blackwell$$c2026
000284047 3367_ $$2DRIVER$$aarticle
000284047 3367_ $$2DataCite$$aOutput Types/Journal article
000284047 3367_ $$0PUB:(DE-HGF)16$$2PUB:(DE-HGF)$$aJournal Article$$bjournal$$mjournal$$s1769070089_9321$$xReview Article
000284047 3367_ $$2BibTeX$$aARTICLE
000284047 3367_ $$2ORCID$$aJOURNAL_ARTICLE
000284047 3367_ $$00$$2EndNote$$aJournal Article
000284047 520__ $$aTDP-linked proteinopathies, including amyotrophic lateral sclerosis (ALS), frontotemporal dementia (FTD) and limbic-predominant age-related TDP-43 encephalopathy (LATE), are characterised by pathogenic deposits containing transactive response DNA-binding protein 43 (TDP-43) in the brain and spinal cord of patients. These hallmark pathological features are associated with widespread neuronal dysfunction and progressive neurodegeneration. TDP-43's role as an essential RNA/DNA-binding protein in RNA metabolism and gene expression regulation is clear, but deciphering the intricate pathophysiological mechanisms underpinning TDP-43-mediated neurodegeneration is paramount for developing effective therapies and novel diagnostic tools for early detection before frank neuronal loss occurs. The nematode Caenorhabditis elegans, with highly conserved TDP-43 orthologue TDP-1, serves as a powerful genetic model to investigate the molecular underpinnings of TDP-43 proteinopathies. Here, we provide a brief overview of the structural and functional characteristics of TDP-43 and TDP-1, highlighting their conserved roles in RNA metabolism, stress responses, and neurodegeneration. We then delve into the pathobiology of TDP-43, drawing insights from C. elegans models expressing either monogenic TDP-43 variants or bigenic combinations with ALS-associated risk genes, and discuss how these models have advanced our understanding of the pathomechanisms of TDP-43 proteinopathies. By employing its simplicity and genetic manipulability, we discuss how these models have helped identify chemical and genetic suppressors of TDP-43-induced phenotypes, including small molecules like Pimozide and the probiotic Lacticaseibacillus rhamnosus HA-114, now in clinical trials. This review underscores the translational value of C. elegans in unraveling the biochemical pathways and interactions in TDP-43 proteinopathies that perturb cellular physiology, potentially facilitating mechanism-based therapy development.
000284047 536__ $$0G:(DE-HGF)POF4-899$$a899 - ohne Topic (POF4-899)$$cPOF4-899$$fPOF IV$$x0
000284047 588__ $$aDataset connected to CrossRef, PubMed, , Journals: pub.dzne.de
000284047 650_7 $$2Other$$aAlzheimer's disease (AD)
000284047 650_7 $$2Other$$aC. elegans
000284047 650_7 $$2Other$$aGABA
000284047 650_7 $$2Other$$aG‐protein coupled receptors
000284047 650_7 $$2Other$$aHuntington's disease
000284047 650_7 $$2Other$$aParkinson's disease (PD)
000284047 650_7 $$2Other$$aTDP‐43/TDP‐1
000284047 650_7 $$2Other$$aacetylcholine
000284047 650_7 $$2Other$$aamyotrophic lateral sclerosis (ALS)
000284047 650_7 $$2Other$$aextracellular vesicles (EV)
000284047 650_7 $$2Other$$afrontotemporal dementia (FTD)
000284047 650_7 $$2Other$$aion channels
000284047 650_7 $$2Other$$alimbic‐predominant age‐related TDP‐43 encephalopathy (LATE)
000284047 650_7 $$2Other$$aproteinopathies
000284047 650_7 $$2Other$$atau
000284047 650_7 $$2NLM Chemicals$$aDNA-Binding Proteins
000284047 650_7 $$2NLM Chemicals$$aCaenorhabditis elegans Proteins
000284047 650_7 $$2NLM Chemicals$$aTARDBP protein, human
000284047 650_2 $$2MeSH$$aAnimals
000284047 650_2 $$2MeSH$$aCaenorhabditis elegans: genetics
000284047 650_2 $$2MeSH$$aCaenorhabditis elegans: metabolism
000284047 650_2 $$2MeSH$$aTDP-43 Proteinopathies: genetics
000284047 650_2 $$2MeSH$$aTDP-43 Proteinopathies: pathology
000284047 650_2 $$2MeSH$$aTDP-43 Proteinopathies: metabolism
000284047 650_2 $$2MeSH$$aDNA-Binding Proteins: genetics
000284047 650_2 $$2MeSH$$aDNA-Binding Proteins: metabolism
000284047 650_2 $$2MeSH$$aDNA-Binding Proteins: chemistry
000284047 650_2 $$2MeSH$$aDisease Models, Animal
000284047 650_2 $$2MeSH$$aHumans
000284047 650_2 $$2MeSH$$aCaenorhabditis elegans Proteins: genetics
000284047 650_2 $$2MeSH$$aCaenorhabditis elegans Proteins: metabolism
000284047 650_2 $$2MeSH$$aNeurodegenerative Diseases: genetics
000284047 650_2 $$2MeSH$$aNeurodegenerative Diseases: pathology
000284047 650_2 $$2MeSH$$aNeurodegenerative Diseases: metabolism
000284047 650_2 $$2MeSH$$aAmyotrophic Lateral Sclerosis: genetics
000284047 650_2 $$2MeSH$$aAmyotrophic Lateral Sclerosis: pathology
000284047 650_2 $$2MeSH$$aAmyotrophic Lateral Sclerosis: metabolism
000284047 650_2 $$2MeSH$$aFrontotemporal Dementia: genetics
000284047 650_2 $$2MeSH$$aFrontotemporal Dementia: pathology
000284047 650_2 $$2MeSH$$aFrontotemporal Dementia: metabolism
000284047 7001_ $$aBuddenkotte, Joerg$$b1
000284047 7001_ $$aAlam, Majid Ali$$b2
000284047 7001_ $$aOwn, Ahmed$$b3
000284047 7001_ $$00000-0003-0461-7721$$aEck, Randall J$$b4
000284047 7001_ $$00000-0002-2252-7634$$aKraemer, Brian C$$b5
000284047 7001_ $$0P:(DE-2719)2541671$$aMandelkow, Eckhard$$b6$$udzne
000284047 7001_ $$00000-0002-7090-2187$$aSteinhoff, Martin$$b7
000284047 773__ $$0PERI:(DE-600)2172518-4$$a10.1111/febs.70239$$gVol. 293, no. 2, p. 348 - 384$$n2$$p348 - 384$$tThe FEBS journal$$v293$$x0014-2956$$y2026
000284047 8564_ $$uhttps://pub.dzne.de/record/284047/files/DZNE-2026-00082.pdf$$yRestricted
000284047 8564_ $$uhttps://pub.dzne.de/record/284047/files/DZNE-2026-00082.pdf?subformat=pdfa$$xpdfa$$yRestricted
000284047 9101_ $$0I:(DE-588)1065079516$$6P:(DE-2719)2541671$$aDeutsches Zentrum für Neurodegenerative Erkrankungen$$b6$$kDZNE
000284047 9131_ $$0G:(DE-HGF)POF4-899$$1G:(DE-HGF)POF4-890$$2G:(DE-HGF)POF4-800$$3G:(DE-HGF)POF4$$4G:(DE-HGF)POF$$aDE-HGF$$bProgrammungebundene Forschung$$lohne Programm$$vohne Topic$$x0
000284047 915__ $$0StatID:(DE-HGF)3001$$2StatID$$aDEAL Wiley$$d2025-01-03$$wger
000284047 915__ $$0StatID:(DE-HGF)0300$$2StatID$$aDBCoverage$$bMedline$$d2025-01-03
000284047 915__ $$0StatID:(DE-HGF)0200$$2StatID$$aDBCoverage$$bSCOPUS$$d2025-01-03
000284047 915__ $$0StatID:(DE-HGF)0600$$2StatID$$aDBCoverage$$bEbsco Academic Search$$d2025-01-03
000284047 915__ $$0StatID:(DE-HGF)0030$$2StatID$$aPeer Review$$bASC$$d2025-01-03
000284047 915__ $$0StatID:(DE-HGF)0199$$2StatID$$aDBCoverage$$bClarivate Analytics Master Journal List$$d2025-01-03
000284047 915__ $$0StatID:(DE-HGF)1050$$2StatID$$aDBCoverage$$bBIOSIS Previews$$d2025-01-03
000284047 915__ $$0StatID:(DE-HGF)0160$$2StatID$$aDBCoverage$$bEssential Science Indicators$$d2025-01-03
000284047 915__ $$0StatID:(DE-HGF)1030$$2StatID$$aDBCoverage$$bCurrent Contents - Life Sciences$$d2025-01-03
000284047 915__ $$0StatID:(DE-HGF)1190$$2StatID$$aDBCoverage$$bBiological Abstracts$$d2025-01-03
000284047 915__ $$0StatID:(DE-HGF)0113$$2StatID$$aWoS$$bScience Citation Index Expanded$$d2025-01-03
000284047 915__ $$0StatID:(DE-HGF)0150$$2StatID$$aDBCoverage$$bWeb of Science Core Collection$$d2025-01-03
000284047 9201_ $$0I:(DE-2719)1013014$$kAG (Eckhard) Mandelkow$$lStructural Principles of Neurodegeneration$$x0
000284047 980__ $$ajournal
000284047 980__ $$aEDITORS
000284047 980__ $$aVDBINPRINT
000284047 980__ $$aI:(DE-2719)1013014
000284047 980__ $$aUNRESTRICTED