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https://www.readbyqxmd.com/read/28208729/altered-intracellular-milieu-of-adar2-deficient-motor-neurons-in-amyotrophic-lateral-sclerosis
#1
REVIEW
Takenari Yamashita, Megumi Akamatsu, Shin Kwak
Transactive response DNA-binding protein (TDP-43) pathology, and failure of A-to-I conversion (RNA editing) at the glutamine/arginine (Q/R) site of α-amino-3-hydroxy-5-methyl-4-isoxazole propionic acid (AMPA) receptor subunit GluA2, are etiology-linked molecular abnormalities that concomitantly occur in the motor neurons of most patients with amyotrophic lateral sclerosis (ALS). Adenosine deaminase acting on RNA 2 (ADAR2) specifically catalyzes GluA2 Q/R site-RNA editing. Furthermore, conditional ADAR2 knockout mice (AR2) exhibit a progressive ALS phenotype with TDP-43 pathology in the motor neurons, which is the most reliable pathological marker of ALS...
February 8, 2017: Genes
https://www.readbyqxmd.com/read/28205009/mixed-pathologies-including-chronic-traumatic-encephalopathy-account-for-dementia-in-retired-association-football-soccer-players
#2
Helen Ling, Huw R Morris, James W Neal, Andrew J Lees, John Hardy, Janice L Holton, Tamas Revesz, David D R Williams
In retired professional association football (soccer) players with a past history of repetitive head impacts, chronic traumatic encephalopathy (CTE) is a potential neurodegenerative cause of dementia and motor impairments. From 1980 to 2010, 14 retired footballers with dementia were followed up regularly until death. Their clinical data, playing career, and concussion history were prospectively collected. Next-of-kin provided consent for six to have post-mortem brain examination. Of the 14 male participants, 13 were professional and 1 was a committed amateur...
February 15, 2017: Acta Neuropathologica
https://www.readbyqxmd.com/read/28197178/tdp-43-overexpression-impairs-presynaptic-integrity
#3
Lanier Heyburn, Charbel E-H Moussa
No abstract text is available yet for this article.
December 2016: Neural Regeneration Research
https://www.readbyqxmd.com/read/28181891/-chronic-traumatic-encephalopathy-an-old-acquaintance-in-athletes
#4
E G B Vijverberg, A C M Pijnenburg, P Scheltens, Y A L Pijnenburg
- Chronic traumatic encephalopathy (CTE) is a neurodegenerative disease caused by repetitive head injuries like those seen in sports such as boxing, American football and soccer.- The clinical features of CTE are a range of cognitive, psychiatric and motor symptoms, and histopathology involves deposits of hyperphosphorylated tau protein and the presence of TAR DNA-binding protein (TDP-43) with relatively little beta-amyloid.- CTE is difficult to differentiate clinically from Alzheimer's disease, frontotemporal dementia and psychiatric disorders because of the major symptom overlap between these conditions...
2017: Nederlands Tijdschrift Voor Geneeskunde
https://www.readbyqxmd.com/read/28176659/intrinsically-disordered-domains-amyloids-and-protein-liquid-phases-evolving-concepts-and-open-questions
#5
Miguel Ángel Mompeán, Douglas Vinson Laurents
Enzymes and structural proteins dominated thinking about protein structure and function for most of the twentieth century. In recent decades, however, we have begun to appreciate the significant physiological and pathological roles of nonglobular proteins. Amyloids first gained infamy from their implications in a score of human mortal diseases. However, they have recently been discovered to play vital physiological roles, such as memory consolidation in humans. This raises an important question: Can we inhibit pathological amyloids without affecting functional amyloids? Intrinsically disordered proteins (IDPs), many of which are prone to form amyloids, perform many essential functions, yet their importance has only been recognized in the last quarter century...
February 6, 2017: Protein and Peptide Letters
https://www.readbyqxmd.com/read/28172957/reduced-stress-granule-formation-and-cell-death-in-fibroblasts-with-the-a382t-mutation-of-tardbp-gene-evidence-for-loss-of-tdp-43-nuclear-function
#6
Sandro Orrù, Paola Coni, Andrea Floris, Roberto Littera, Carlo Carcassi, Valeria Sogos, Carla Brancia
No abstract text is available yet for this article.
October 15, 2016: Human Molecular Genetics
https://www.readbyqxmd.com/read/28167899/cysteine-modifications-in-the-pathogenesis-of-als
#7
REVIEW
Cristiana Valle, Maria Teresa Carrì
Several proteins are found misfolded and aggregated in sporadic and genetic forms of amyotrophic lateral sclerosis (ALS). These include superoxide dismutase (SOD1), transactive response DNA-binding protein (TDP-43), fused in sarcoma/translocated in liposarcoma protein (FUS/TLS), p62, vasolin-containing protein (VCP), Ubiquilin-2 and dipeptide repeats produced by unconventional RAN-translation of the GGGGCC expansion in C9ORF72. Up to date, functional studies have not yet revealed a common mechanism for the formation of such diverse protein inclusions...
2017: Frontiers in Molecular Neuroscience
https://www.readbyqxmd.com/read/28167528/heat-shock-induced-phosphorylation-of-tar-dna-binding-protein-43-tdp-43-by-mapk-erk-kinase-regulates-tdp-43-function
#8
Wen Li, Ashley N Reeb, Binyan Lin, Praveen Subramanian, Erin E Fey, Catherine R Knoverek, Rachel L French, Eileen H Bigio, Yuna M Ayala
TAR DNA binding protein (TDP-43) is a highly conserved and essential DNA- and RNA-binding protein that controls gene expression through RNA processing, in particular, regulation of splicing. Intracellular aggregation of TDP-43 is a hallmark of amyotrophic lateral sclerosis and ubiquitin-positive frontotemporal lobar degeneration. This TDP-43 pathology is also present in other types of neurodegeneration including Alzheimer's disease. We report here that TDP-43 is a substrate of MEK, a central kinase in the MAPK/ERK signaling pathway...
February 6, 2017: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/28163215/vegf-alleviates-als-csf-induced-cytoplasmic-accumulations-of-tdp-43-and-fus-tls-in-nsc-34-cells
#9
Shubham Shantanu, K Vijayalakshmi, S Shruthi, B K Chandrasekhar Sagar, T N Sathyaprabha, A Nalini, Trichur R Raju, Phalguni Anand Alladi
Cytoplasmic mislocalisation and aggregation of TDP-43 and FUS/TLS proteins in spinal motor neurons contribute to the pathogenesis of the highly fatal disorder amyotrophic lateral sclerosis (ALS). We investigated the neuroprotective effect of VEGF on expression of these proteins in the motor neuronal cell line NSC-34 modelled to reminisce sporadic form of ALS. We studied the expression of TDP-43 and FUS/TLS proteins after exposure to ALS-CSF and following VEGF supplementation by quantitative confocal microscopy and electron microscopy...
February 2, 2017: Journal of Chemical Neuroanatomy
https://www.readbyqxmd.com/read/28160067/tau-aggregation-influences-cognition-and-hippocampal-atrophy-in-the-absence-of-beta-amyloid-a-clinico-imaging-pathological-study-of-primary-age-related-tauopathy-part
#10
Keith A Josephs, Melissa E Murray, Nirubol Tosakulwong, Jennifer L Whitwell, David S Knopman, Mary M Machulda, Stephen D Weigand, Bradley F Boeve, Kejal Kantarci, Leonard Petrucelli, Val J Lowe, Clifford R Jack, Ronald C Petersen, Joseph E Parisi, Dennis W Dickson
We investigate whether there is any association between the Braak neurofibrillary tangle (NFT) stage and clinical and MRI features in definite primary age-related tauopathy (PART). We analysed 52 cases with a Braak NFT tangle stage >0 and ≤IV, and a Thal phase of 0 (no beta-amyloid present). Twenty-nine (56%) were female. Median age at death was 88 years (IQR 82-92 years). Fifteen (29%) were TDP-positive (75% TDP stage I), 16 (31%) had argyrophilic grain disease and three (6%) had alpha-synuclein-positive Lewy bodies...
February 3, 2017: Acta Neuropathologica
https://www.readbyqxmd.com/read/28158562/increased-cytoplasmic-tdp-43-reduces-global-protein-synthesis-by-interacting-with-rack1-on-polyribosomes
#11
Russo Arianna, Scardigli Raffaella, La Regina Federico, Murray E Melissa, Romano Nicla, Dickson W Dennis, Wolozin Benjamin, Cattaneo Antonino, Ceci Marcello
No abstract text is available yet for this article.
February 1, 2017: Human Molecular Genetics
https://www.readbyqxmd.com/read/28155653/novel-group-based-qsar-and-combinatorial-design-of-ck-1%C3%AE-inhibitors-as-neuroprotective-agents
#12
Kopal Joshi, Sukriti Goyal, Sonam Grover, Salma Jamal, Aditi Singh, Pawan Dhar, Abhinav Grover
BACKGROUND: Tar DNA binding protein 43 (TDP-43) hyperphosphorylation, caused by Casein kinase 1 (CK-1) protein isoforms, is associated with the onset and progression of Amyotrophic Lateral Sclerosis (ALS). Among the reported isoforms and splice variants of CK-1 protein superfamily, CK-1δ is known to phosphorylate different serine and threonine sites on TDP-43 protein in vitro and thus qualifies as a potential target for ALS treatment. RESULTS: The developed GQSAR (group based quantitative structure activity relationship) model displayed satisfactory statistical parameters for the dataset of experimentally reported N-Benzothiazolyl-2-Phenyl Acetamide derivatives...
December 22, 2016: BMC Bioinformatics
https://www.readbyqxmd.com/read/28153034/tdp-43-cryptic-exons-are-highly-variable-between-cell-types
#13
Yun Ha Jeong, Jonathan P Ling, Sophie Z Lin, Aneesh N Donde, Kerstin E Braunstein, Elisa Majounie, Bryan J Traynor, Katherine D LaClair, Thomas E Lloyd, Philip C Wong
BACKGROUND: TDP-43 proteinopathy is a prominent pathological feature that occurs in a number of human diseases including amyotrophic lateral sclerosis (ALS), frontotemporal dementia (FTD), and inclusion body myositis (IBM). Our recent finding that TDP-43 represses nonconserved cryptic exons led us to ask whether cell type-specific cryptic exons could exist to impact unique molecular pathways in brain or muscle. METHODS: In the present work, we investigated TDP-43's function in various mouse tissues to model disease pathogenesis...
February 2, 2017: Molecular Neurodegeneration
https://www.readbyqxmd.com/read/28139767/drosophila-cg3303-is-an-essential-endoribonuclease-linked-to-tdp-43-mediated-neurodegeneration
#14
Pietro Laneve, Lucia Piacentini, Assunta Maria Casale, Davide Capauto, Ubaldo Gioia, Ugo Cappucci, Valerio Di Carlo, Irene Bozzoni, Patrizio Di Micco, Veronica Morea, Carmela Antonia Di Franco, Elisa Caffarelli
Endoribonucleases participate in almost every step of eukaryotic RNA metabolism, acting either as degradative or biosynthetic enzymes. We previously identified the founding member of the Eukaryotic EndoU ribonuclease family, whose components display unique biochemical features and are flexibly involved in important biological processes, such as ribosome biogenesis, tumorigenesis and viral replication. Here we report the discovery of the CG3303 gene product, which we named DendoU, as a novel family member in Drosophila...
January 31, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28130640/expansion-of-the-classification-of-ftld-tdp-distinct-pathology-associated-with-rapidly-progressive-frontotemporal-degeneration
#15
Edward B Lee, Sílvia Porta, G Michael Baer, Yan Xu, EunRan Suh, Linda K Kwong, Lauren Elman, Murray Grossman, Virginia M-Y Lee, David J Irwin, Vivianna M Van Deerlin, John Q Trojanowski
Frontotemporal lobar degeneration with TDP-43 inclusions (FTLD-TDP) can typically be categorized into one of four distinct histopathologic patterns of TDP-43 pathology, types A to D. The strength of this histopathologic classification lies in the association between FTLD-TDP subtypes and various clinical and genetic features of disease. Seven cases of FTLD-TDP were identified here which were difficult to classify based on existing pathologic criteria. Distinct features common to these cases included TDP-43 aggregates over a wide neuroanatomic distribution comprised of granulofilamentous neuronal inclusions, abundant grains, and oligodendroglial inclusions...
January 27, 2017: Acta Neuropathologica
https://www.readbyqxmd.com/read/28129109/motor-coordinative-and-cognitive-dysfunction-caused-by-mutant-tdp-43-could-be-reversed-by-inhibiting-its-mitochondrial-localization
#16
Wenzhang Wang, Hiroyuki Arakawa, Luwen Wang, Ogoegbunam Okolo, Sandra L Siedlak, Yinfei Jiang, Ju Gao, Fei Xie, Robert B Petersen, Xinglong Wang
Dominant missense mutations in TAR DNA-binding protein 43 (TDP-43) cause amyotrophic lateral sclerosis (ALS), and the cytoplasmic accumulation of TDP-43 represents a pathological hallmark in ALS and frontotemporal lobar degeneration (FTD). Behavioral investigation of the transgenic mouse model expressing the disease-causing human TDP-43 M337V mutant (TDP-43(M337V) mice) is encumbered by premature death in homozygous transgenic mice and a reported lack of phenotype assessed by tail elevation and footprint in hemizygous transgenic mice...
January 4, 2017: Molecular Therapy: the Journal of the American Society of Gene Therapy
https://www.readbyqxmd.com/read/28129105/new-therapeutic-avenue-for-als-avoiding-a-fatal-encounter-of-tdp-43-at-the-mitochondria
#17
Eloise Hudry
No abstract text is available yet for this article.
January 4, 2017: Molecular Therapy: the Journal of the American Society of Gene Therapy
https://www.readbyqxmd.com/read/28128723/white-matter-pathology-in-sporadic-frontotemporal-lobar-degeneration-with-tdp-43-proteinopathy
#18
Richard A Armstrong
AIMS: To characterize white matter pathology in frontotemporal lobar degeneration (FTLD) with TDP-43 proteinopathy (FTLD-TDP) and its relationship to gray matter pathology. MATERIAL: Fiber tracts from frontal and temporal lobes of 10 sporadic cases of FTLD and 8 controls. METHOD: Density and spatial patterns of vacuolation, glial cell nuclei, and glial inclusions (GI) were studied in 4 fiber tracts from each case. RESULTS: Densities of vacuoles but not glial cells were greater in FTLD-TDP than controls...
January 27, 2017: Clinical Neuropathology
https://www.readbyqxmd.com/read/28126008/elevated-tmem106b-levels-exaggerate-lipofuscin-accumulation-and-lysosomal-dysfunction-in-aged-mice-with-progranulin-deficiency
#19
Xiaolai Zhou, Lirong Sun, Owen Adam Brady, Kira A Murphy, Fenghua Hu
Mutations resulting in haploinsufficiency of progranulin (PGRN) cause frontotemporal lobar degeneration with TDP-43-positive inclusions (FTLD-TDP), a devastating neurodegenerative disease. Accumulating evidence suggest a crucial role of progranulin in maintaining proper lysosomal function during aging. TMEM106B has been identified as a risk factor for frontotemporal lobar degeneration with progranulin mutations and elevated mRNA and protein levels of TMEM106B are associated with increased risk for frontotemporal lobar degeneration...
January 26, 2017: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/28122516/tdp-43-protein-variants-as-biomarkers-in-amyotrophic-lateral-sclerosis
#20
Stephanie M Williams, Galam Khan, Brent T Harris, John Ravits, Michael R Sierks
BACKGROUND: TDP-43 aggregates accumulate in individuals affected by amyotrophic lateral sclerosis (ALS) and other neurodegenerative diseases, representing potential diagnostic and therapeutic targets. Using an atomic force microscopy based biopanning protocol developed in our lab, we previously isolated 23 TDP-43 reactive antibody fragments with preference for human ALS brain tissue relative to frontotemporal dementia, a related neurodegeneration, and healthy samples from phage-displayed single chain antibody fragment (scFv) libraries...
January 25, 2017: BMC Neuroscience
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