20 papers · ranked by Valyu relevance
Chunhui Huang, Sen Yan, Zaijun Zhang
Mitochondria are the energy center of cell operations and are involved in physiological functions and maintenance of metabolic balance and homeostasis in the body. Alterations of mitochondrial function are associated with a variety of degenerative and acute diseases. As mitochondria age in cells, they gradually become…
Manuela Neumann
The identification of TDP-43 as the major component of the pathologic inclusions in most forms of sporadic and familial frontotemporal lobar degeneration with ubiquitin-positive inclusions (FTLD-U) and amyotrophic lateral sclerosis (ALS) resolved a long-standing enigma concerning the nature of the ubiquitinated disease…
Fiona Bright, Gabriella Chan, Annika van Hummel, Lars M. Ittner + 2 more
'Yazi D. Ke' 'Johannes Berger'] The abnormal mislocalisation and ubiquitinated protein aggregation of the TAR DNA binding protein 43 (TDP-43) within the cytoplasm of neurons and glia in the central nervous system (CNS) is a pathological hallmark of early-onset neurodegenerative disorders amyotrophic lateral sclerosis…
Wendi Huang, Yongjian Zhou, Lin Tu, Zhisheng Ba + 3 more
'Nanqu Huang' 'Yong Luo'] Since the discovery of TAR DNA-binding protein 43 (TDP-43) in 1995, our understanding of its role continues to expand as research progresses. In particular, its role in the pathogenesis of Alzheimer’s disease (AD) has drawn increasing interest in recent years. TDP-43 may participate in various…
Liberty Francois-Moutal, David Donald Scott, May Khanna
Tar DNA binding (TDP)-43 proteinopathy, typically described as cytoplasmic accumulation of highly modified and misfolded TDP-43 molecules, is characteristic of several neurodegenerative diseases such as Amyotrophic Lateral Sclerosis (ALS) and limbic-predominant age-related TDP-43 encephalopathy (LATE). TDP-43 proposed…
Yara Al Ojaimi, Rudolf Hergesheimer, Anna A. Chami, Hugo Alarcan + 14 more
TDP-43 proteinopathies are a set of neurological disorders characterized by the abnormal accumulation and mislocalization of TDP-43 in the cytoplasm, leading to the disruption of the normal function of the protein. In most of the cases, it is the wildtype (wt) form of the protein that is involved. An untargeted…
Anukool A. Bhopatkar, Vladimir N. Uversky, Vijayaraghavan Rangachari
Tar DNA binding protein (TDP-43) has emerged as a key player in many neurodegenerative pathologies including frontotemporal lobar degeneration (FTLD) and amyotropic lateral sclerosis (ALS). Important hallmarks of FTLD and ALS are the toxic cytoplasmic inclusions of C-terminal fragments of TDP-43 (TDP-43CTD), which are…
Beibei Zhao, Sarah Louadi, Juliane A. Coutts, Ebrima Gibbs + 11 more
TAR DNA-binding protein 43 (TDP-43), encoded by the TARDBP gene, is a ribonucleoprotein associated with the pathogenesis of amyotrophic lateral sclerosis (ALS), frontotemporal dementia (FTD), and Alzheimer’s disease (AD). Under physiological conditions, TDP-43 is predominantly localized in the nucleus, where it…
Tanzeen Yusuff, Shreyasi Chatterjee, Ya-Chu Chang, Tzu-Kang Sang + 1 more
Transactive response DNA binding protein-43 (TDP-43) is known to mediate neurodegeneration associated with amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD). The exact mechanism by which TDP-43 exerts toxicity in the brains of affected patients remains unclear. In a novel Drosophila…
Florencia Vassallu, Milagros López, Florencia López Ambrosioni, Juan Casal + 2 more
TDP-43 proteinopathy is the primary pathology associated with amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD), indicating that these neurodegenerative diseases have common underlying mechanisms. We have previously shown that transgenic (Tg) mice conditionally overexpressing a cytoplasmic form of…
Alexander Muacevic, John R Adler, Hussain Hussain, Tamara Djurin + 5 more
'Jacqueline Rodriguez' 'Lia Daneelian' 'Sardar Sundi' 'Aya Fadel' 'Zahraa Saadoon'] The trans-activation response DNA-binding protein of 43kDa (TDP-43) is involved in the pathogenesis of multiple brain disorders. As scientists are unraveling TDP-43 function and its impact on various diseases, we have begun to…
Muthu Raj Salaikumaran, Pallavi P. Gopal
TDP-43, an essential RNA/DNA-binding protein, is central to the pathology of neurodegenerative diseases such as Amyotrophic Lateral Sclerosis and Frontotemporal Dementia. Pathological mislocalization and aggregation of TDP-43 disrupts RNA splicing, mRNA stability, and mRNA transport, thereby impairing neuronal function…
Yui Matsushita, Ikki Yasuda, Fuga Watanabe, Eiji Yamamoto
RNA-binding proteins form biomolecular condensates with RNA through phase separation, playing crucial roles in various cellular processes. While intrinsically disordered regions (IDRs) are key drivers of phase separation, additional factors such as folded domains and RNA also influence condensate formation and physical…
Greta Grassmann, Mattia Miotto, Lorenzo Di Rienzo, Federico Salaris + 6 more
'Beatrice Silvestri' 'Elsa Zacco' 'Alessandro Rosa' 'Gian Gaetano Tartaglia' 'G. Ruocco' 'Edoardo Milanetti'] Many of the molecular mechanisms underlying the pathological aggregation of proteins observed in neurodegenerative diseases are still not fully understood. Among the diseases associated with protein aggregates…
Anukool A. Bhopatkar, Shailendra Dhakal, Vijayaraghavan Rangachari
Cytoplasmic inclusions containing aberrant proteolytic fragments of TDP-43 are associated with frontotemporal lobar degeneration (FTLD) and other related pathologies. In FTLD, TDP-43 is translocated into the cytoplasm and proteolytically cleaved to generate a prion-like domain (PrLD) containing C-terminal fragments…
Rumiana Tenchov, Janet Sasso, Qiongqiong Angela Zhou
Alzheimer's disease (AD) is a progressive neurodegenerative disorder characterized by cognitive decline, memory loss, and impaired daily functioning. The pathology of AD is marked by the accumulation of amyloid-beta plaques and tau protein tangles in brain, along with neuroinflammation and synaptic dysfunction. Genetic…
Vincent Huin, Mathieu Barbier, Armand Bottani, Johannes Alexander Lobrinus + 17 more
'Johannes Alexander Lobrinus' 'Fabienne Clot' 'Foudil Lamari' 'Laureen Chat' 'Benoît Rucheton' 'Frédérique Fluchère' 'Stéphane Auvin' 'Peter L. Myers' 'A. Gélot' 'Agnès Camuzat' 'Catherine Caillaud' 'Ludmila Jornéa' 'Sylvie Forlani' 'Dario Saracino' 'Charles Duyckaerts' 'Alexis Brice' 'Alexandra Dürr' 'Isabelle Le…
P. G. Kevrekidis, Travis Thompson, Alain Goriely
Neurodegenerative diseases are closely associated with the amplification and invasion of toxic proteins. In particular Alzheimer's disease is characterized by the systematic progression of amyloid-β and τ-proteins in the brain. These two protein families are coupled and it is believed that their joint presence greatly…
Satomi Imaide, Kristin M. Riching, Vesna Vetma, Claire Whitworth + 10 more
Bivalent small-molecule degraders, or proteolysis targeting chimeras (PROTACs), work by simultaneously binding a target protein and E3 ubiquitin ligase to produce a ternary complex. To drive target ubiquitination and degradation at low catalytic concentrations, degraders must form appropriately positioned complexes of…
Swadesh Pal, Roderick Melnik
It is well known that today nearly one in six of the world's population has to deal with neurodegenerative disorders. While a number of medical devices have been developed for the detection, prevention, and treatments of such disorders, some fundamentals of the progression of associated diseases are in urgent need of…