Archana Prasad†, Vidhya Bharathi†, Vishwanath Sivalingam, Amandeep Girdhar and Basant K. Patel* Department of Biotechnology, Indian Institute of Technology Hyderabad, Sangareddy, India TAR DNA binding protein 43 (TDP-43) is a versatile RNA/DNA binding protein involved in
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As well as ALS (amyotrophic lateral sclerosis) and FTLD (frontotemporal lobar degeneration), mutations in TDP-43 have also been associated Parkinson's
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TAR DNA binding protein 43 (TDP-43) Molecular mechanisms of TDP-43 misfolding and pathology in amyotrophic lateral sclerosis. Prasad A; Bharathi V; Sivalingam V; et al. See more; Frontiers in Molecular Neuroscience. DOI: 10.3389/fnmol.2019.00025. 147 Citations. Citations of
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2014. 12. 9. · Amyotrophic lateral sclerosis These mechanisms could be a plausible molecular basis for this disease continuum and, This misfolded conformation then recruits native monomers of TDP-43 to induce pathological misfolding on to the native form in what is known as ‘templated conformational change’.
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Amyotrophic lateral sclerosis (ALS) is a rapidly progressive neurodegenerative disorder involving loss of upper and lower motor neurons, with most cases ending in death within 3-5 years of onset. Several molecular and cellular pathways have been identified to cause ALS; however, treatments to stop or reverse disease progression are yet to be found. Riluzole, a neuroprotective agent offering
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Introduction. Jean-Martin Charcot (1825-1893), in his comments of 1887, underestimated the complexity of the neurodegenerative disease he named "la sclérose amyotrophique": The diagnosis as well as the anatomy and physiology of the condition amyotrophic lateral sclerosis is one of the most completely understood conditions in the realm of clinical neurology.
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Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease involving the formation of cytoplasmic aggregates by proteins including TDP-43 and SOD1, in affected cells in the central nervous system (CNS). Pathology spreads from an initial site of onset to contiguous anatomical regions.
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Over the last two decades, the pathogenic aggregation of TAR DNA-binding protein 43 (TDP-43) is found to be strongly associated with several
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Even though the exact mechanisms remain largely unknown, pathological TDP-43 is thought to exert a plethora of deleterious effects ranging from
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TDP-43 has versatile functions and it is involved in several steps of RNA metabolism such as: transcription, translation, mRNA transport, mRNA stabilization, microRNA (miRNA) and long non-coding
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Prasad, A., Bharathi, V., Sivalingam, V., Girdhar, A., & Patel, B. K. ( ). Molecular Mechanisms of TDP-43 Misfolding and Pathology in Amyotrophic Lateral Sclerosis
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2019. 2. 14. · TAR DNA binding protein 43 (TDP-43) is a versatile RNA/DNA binding protein involved in RNA-related metabolism. Hyper-phosphorylated and ubiquitinated TDP-43 deposits
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Molecular Mechanisms of TDP-43 Misfolding and Pathology in Amyotrophic Lateral Sclerosis. Frontiers in Molecular Neuroscience ( IF 5.639 ) Pub Date
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Neuropathology. A total of 97 autopsy cases between 36 and 98 years of age (mean age: 72 years old, 45 females and 52 males) were investigated: 20 non-diseased controls, 16 pre-clinical AD, 51 neuropathologically-confirmed AD cases and 10 FTLD-TDP cases as positive controls for TDP-43 pathology (Table 1, Additional file 1-Table A1).). Cases with hippocampal sclerosis were not
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Formation of TDP-43 pathology is a distinguishing feature in a wide range of neurodegenerative disorders including FTLD and ALS disorders, and to a lesser
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TAR DNA binding protein 43 (TDP-43) is a versatile RNA/DNA binding protein involved in RNA-related metabolism. Molecular mechanisms of TDP-43 misfolding and pathology in amyotrophic lateral sclerosis. Prasad A; Bharathi V; Sivalingam V; et al. See more; Frontiers in Molecular Neuroscience. DOI: 10.3389/fnmol.2019.00025. 147 Citations
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