Combined Pathologies in FTLD-TDP Types A and C
نویسندگان
چکیده
منابع مشابه
ALS and FTLD: two faces of TDP-43 proteinopathy.
Major discoveries have been made in the recent past in the genetics, biochemistry and neuropathology of frontotemporal lobar degeneration (FTLD). TAR DNA-binding protein 43 (TDP-43), encoded by the TARDBP gene, has been identified as the major pathological protein of FTLD with ubiquitin-immunoreactive (ub-ir) inclusions (FTLD-U) with or without amyotrophic lateral sclerosis (ALS) and sporadic A...
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abstract this study investigates the teachers’ correction of students’ spoken errors of linguistic forms in efl classes, aiming at (a) examining the relationship between the learners’ proficiency level and the provision of corrective feedback types, (b) exploring the extent to which teachers’ use of different corrective feedback types is related to the immediate types of context in which err...
Investigating the role of filamin C in Belgian patients with frontotemporal dementia linked to GRN deficiency in FTLD-TDP brains
TAR DNA-binding protein 43 (TDP-43) inclusions are pathological hallmarks of patients with frontotemporal lobar degeneration (FTLD) and amyotrophic lateral sclerosis (ALS). Loss of TDP-43 in zebrafish engenders a severe muscle and vascular phenotype with a concomitant elevation of filamin C (FLNC) levels, an observation confirmed in the frontal cortex of FTLD-TDP patients. Here, we aimed to fur...
متن کاملThe role of TDP-43 in the pathogenesis of ALS and FTLD.
TDP-43 (TAR DNA-binding protein 43) is an hnRNP (heterogeneous nuclear ribonucleoprotein) protein whose role in cellular processes has come to the forefront of neurodegeneration research after the observation that it is the main component of brain inclusions in ALS (amyotrophic lateral sclerosis) and FTLD (frontotemporal lobar degeneration) patients. Functionally, this aberrant aggregation and ...
متن کاملEarly retinal neurodegeneration and impaired Ran-mediated nuclear import of TDP-43 in progranulin-deficient FTLD
Frontotemporal dementia (FTD) is the most common cause of dementia in people under 60 yr of age and is pathologically associated with mislocalization of TAR DNA/RNA binding protein 43 (TDP-43) in approximately half of cases (FLTD-TDP). Mutations in the gene encoding progranulin (GRN), which lead to reduced progranulin levels, are a significant cause of familial FTLD-TDP. Grn-KO mice were develo...
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ژورنال
عنوان ژورنال: Journal of Neuropathology & Experimental Neurology
سال: 2018
ISSN: 0022-3069,1554-6578
DOI: 10.1093/jnen/nly018