Triad of TDP43 control in neurodegeneration: autoregulation, localization and aggregation
P Tziortzouda, L Van Den Bosch, F Hirth - Nature Reviews …, 2021 - nature.com
Cytoplasmic aggregation of TAR DNA-binding protein 43 (TDP43; also known as TARDBP
or TDP-43) is a key pathological feature of several neurodegenerative diseases, including …
or TDP-43) is a key pathological feature of several neurodegenerative diseases, including …
Connecting TDP-43 pathology with neuropathy
JR Klim, G Pintacuda, LA Nash, IG San Juan… - Trends in …, 2021 - cell.com
Transactive response DNA-binding protein 43 kDa (TDP-43), a multifunctional nucleic acid-
binding protein, is a primary component of insoluble aggregates associated with several …
binding protein, is a primary component of insoluble aggregates associated with several …
Nuclear accumulation of CHMP7 initiates nuclear pore complex injury and subsequent TDP-43 dysfunction in sporadic and familial ALS
AN Coyne, V Baskerville, BL Zaepfel… - Science translational …, 2021 - science.org
Alterations in the components [nucleoporins (Nups)] and function of the nuclear pore
complex (NPC) have been implicated as contributors to the pathogenesis of genetic forms of …
complex (NPC) have been implicated as contributors to the pathogenesis of genetic forms of …
[HTML][HTML] Huntington's disease mice and human brain tissue exhibit increased G3BP1 granules and TDP43 mislocalization
II Sanchez, TB Nguyen, WE England… - The Journal of …, 2021 - Am Soc Clin Investig
Chronic cellular stress associated with neurodegenerative disease can result in the
persistence of stress granule (SG) structures, membraneless organelles that form in …
persistence of stress granule (SG) structures, membraneless organelles that form in …
[HTML][HTML] New perspectives on cytoskeletal dysregulation and mitochondrial mislocalization in amyotrophic lateral sclerosis
F Theunissen, PK West, S Brennan, B Petrović… - Translational …, 2021 - Springer
Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease
characterized by selective, early degeneration of motor neurons in the brain and spinal cord …
characterized by selective, early degeneration of motor neurons in the brain and spinal cord …
[HTML][HTML] HnRNP K mislocalisation is a novel protein pathology of frontotemporal lobar degeneration and ageing and leads to cryptic splicing
A Bampton, A Gatt, J Humphrey, S Cappelli… - Acta …, 2021 - Springer
Heterogeneous nuclear ribonucleoproteins (HnRNPs) are a group of ubiquitously
expressed RNA-binding proteins implicated in the regulation of all aspects of nucleic acid …
expressed RNA-binding proteins implicated in the regulation of all aspects of nucleic acid …
[HTML][HTML] An integrated multi-omic analysis of iPSC-derived motor neurons from C9ORF72 ALS patients
Neurodegenerative diseases are challenging for systems biology because of the lack of
reliable animal models or patient samples at early disease stages. Induced pluripotent stem …
reliable animal models or patient samples at early disease stages. Induced pluripotent stem …
[HTML][HTML] Novel STMN2 Variant Linked to Amyotrophic Lateral Sclerosis Risk and Clinical Phenotype
Objective There is a critical need to establish genetic markers that explain the complex
phenotypes and pathogenicity of ALS. This study identified a polymorphism in the Stathmin …
phenotypes and pathogenicity of ALS. This study identified a polymorphism in the Stathmin …
[HTML][HTML] Human induced pluripotent stem cell-derived TDP-43 mutant neurons exhibit consistent functional phenotypes across multiple gene edited lines despite …
Gene editing technologies hold great potential to enhance our ability to model inheritable
neurodegenerative diseases. Specifically, engineering multiple amyotrophic lateral sclerosis …
neurodegenerative diseases. Specifically, engineering multiple amyotrophic lateral sclerosis …
[HTML][HTML] Novel insight into the role of immune dysregulation in amyotrophic lateral sclerosis based on bioinformatic analysis
Y Xie, X Luo, H He, M Tang - Frontiers in Neuroscience, 2021 - frontiersin.org
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disorder characterized by
the progressive degeneration of motor neurons. The causative pathogenic mechanisms in …
the progressive degeneration of motor neurons. The causative pathogenic mechanisms in …