A novel fly model of TDP-43 proteinopathies: N-terminus sequences combined with the Q/N domain induce protein functional loss and locomotion defects
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منابع مشابه
A novel fly model of TDP-43 proteinopathies: N-terminus sequences combined with the Q/N domain induce protein functional loss and locomotion defects
is ea se M o de ls & M ec ha ni sm s • D M M • A dv an ce a rt ic le © 2016. Published by The Company of Biologists Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution and reproduction in any medium provided that the original work is properly attribu...
متن کاملA novel Drosophila model of TDP-43 proteinopathies: N-terminal sequences combined with the Q/N domain induce protein functional loss and locomotion defects
Transactive response DNA-binding protein 43 kDa (TDP-43, also known as TBPH in Drosophila melanogaster and TARDBP in mammals) is the main protein component of the pathological inclusions observed in neurons of patients affected by different neurodegenerative disorders, including amyotrophic lateral sclerosis (ALS) and fronto-temporal lobar degeneration (FTLD). The number of studies investigatin...
متن کاملMolecular Neuropathology of TDP-43 Proteinopathies
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 protein under these conditions. Anti-TDP-43 immunohistochemistry and the rec...
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 کاملClinical and pathological continuum of multisystem TDP-43 proteinopathies.
OBJECTIVE To determine the extent of transactivation response DNA-binding protein with a molecular weight of 43 kDa (TDP-43) pathology in the central nervous system of patients with clinically and autopsy-confirmed diagnoses of frontotemporal lobar degeneration with and without motor neuron disease and amyotrophic lateral sclerosis with and without cognitive impairment. DESIGN Performance of ...
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ژورنال
عنوان ژورنال: Disease Models & Mechanisms
سال: 2016
ISSN: 1754-8411,1754-8403
DOI: 10.1242/dmm.023382