Absence of TDP ‐43 is difficult to digest
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چکیده
منابع مشابه
Absence of TDP-43 is difficult to digest.
It is well established that TDP-43 accumulates in degenerating neurons in patients with ALS/FTLD, which might affect normal TDP-43 function. In this issue of The EMBO Journal Xia et al (2016) show a novel connection between TDP-43 loss of function and autophagy failure. Using knockdown models of TDP-43, they observed enhanced autophagosome and lysosome biogenesis through mTORC1 activity inhibit...
متن کاملDrosophila Answers to TDP-43 Proteinopathies
Initially implicated in the pathogenesis of CFTR and HIV-1 transcription, nuclear factor TDP-43 was subsequently found to be involved in the origin and development of several neurodegenerative diseases. In 2006, in fact, it was reported for the first time the cytoplasmic accumulation of TDP-43 in ubiquitin-positive inclusions of ALS and FTLD patients, suggesting the presence of a shared underly...
متن کاملExpression of TDP-43 C-terminal Fragments in Vitro Recapitulates Pathological Features of TDP-43 Proteinopathies.
The disease protein in frontotemporal lobar degeneration with ubiquitin-positive inclusions (FTLD-U) and amyotrophic lateral sclerosis (ALS) was identified recently as the TDP-43 (TAR DNA-binding protein 43), thereby providing a molecular link between these two disorders. In FTLD-U and ALS, TDP-43 is redistributed from its normal nuclear localization to form cytoplasmic insoluble aggregates. Mo...
متن کاملTDP-43 and frontotemporal dementia.
TAR DNA-binding protein of about 43 kDa (TDP-43) is the main ubiquitinated peptide in tau-negative frontotemporal lobar degeneration (FTLD). TDP-43 is typically a nuclear protein, and its aggregation and cytoplasmic translocation are thought to represent major steps in the pathogenesis of FTLD due to TDP-43 proteinopathy (FTLD-TDP). Certain clinical syndromes of frontotemporal dementia are pref...
متن کاملTDP-43 toxicity in yeast.
The budding yeast Saccharomyces cerevisiae is an emerging tool for investigating the molecular pathways that underpin several human neurodegenerative disorders associated with protein misfolding. Amyotrophic lateral sclerosis (ALS) is a devastating adult onset neurodegenerative disease primarily affecting motor neurons. The protein TDP-43 has recently been demonstrated to play an important role...
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ژورنال
عنوان ژورنال: The EMBO Journal
سال: 2015
ISSN: 0261-4189,1460-2075
DOI: 10.15252/embj.201593603