The DOI matches its Crossref record; 420 of the 420 references it cites were found in a registry or an index.
Molecular Mechanisms of TDP-43 Misfolding and Pathology in Amyotrophic Lateral Sclerosis
Open accessAbstract
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 as inclusion bodies in the brain and spinal cord of the patients with the motor neuron diseases: amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD). While majority of the ALS cases (90-95%) are sporadic (sALS), among the familial ALS cases 5-10% involve the inheritance of mutations in the TARDBP gene and the remaining (90-95%) are due to mutations in other genes such as: C9ORF72, SOD1, FUS, and NEK1 etc. Strikingly however, majority of the sporadic ALS patients (up to 97%) also contain the TDP-43 protein deposited in the neuronal inclusions, which suggests of its pivotal role in the ALS pathology. Thus, unravelling the molecular mechanisms of the TDP-43 pathology, seems central to the ALS therapeutics, hence, we comprehensively review the current understanding of the TDP-43’s pathology in ALS. We discuss the roles of TDP-43’s mutations, its cytoplasmic mis-localization and aberrant post-translational modifications in ALS. Also, we evaluate TDP-43’s amyloid-like in vitro aggregation, its physiological versus pathological oligomerization in vivo, liquid-liquid phase separation (LLPS), and potential prion-like propagation propensity of the TDP-43 inclusions. Finally, we describe the various evolving TDP-43-induced toxicity mechanisms such as the impairment of endocytosis and mitotoxicity etc. and also discuss the emerging strategies towards TDP-43 disaggregation and ALS therapeutics.
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Metadata
- Authors
- Prasad, A., Bharathi, V., Sivalingam, V., Girdhar, A., & Patel, B. K.
- Venue
- Frontiers in Molecular Neuroscience
- Publisher
- Frontiers Media SA
- Published
- 2019-02-14
- Volume
- 12
- Pages
- 25-25
- ISSN
- 1662-5099 (ISSN-L)
- OpenAlex
- W2911237161
- Cited by
- 774
- References
- 420
- FWCI
- 47.43
- Type
- journal article
- Language
- en
- Open access
- gold
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- Prasad, A., Bharathi, V., Sivalingam, V., Girdhar, A., & Patel, B. K. (2019). Molecular Mechanisms of TDP-43 Misfolding and Pathology in Amyotrophic Lateral Sclerosis. Frontiers in Molecular Neuroscience. https://doi.org/10.3389/fnmol.2019.00025
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