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2015 Fiscal Year Final Research Report

Heterogeneity of TDP-43 pathology and clinicopathologic correlations in sporadic amyotrophic lateral sclerosis

Research Project

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Project/Area Number 26640029
Research Category

Grant-in-Aid for Challenging Exploratory Research

Allocation TypeMulti-year Fund
Research Field Nerve anatomy/Neuropathology
Research InstitutionNiigata University

Principal Investigator

TAKAHASHI Hitoshi  新潟大学, 脳研究所, 教授 (90206839)

Co-Investigator(Kenkyū-buntansha) TOYOSHIMA Yasuko  新潟大学, 脳研究所, 准教授 (20334675)
TADA Mari  新潟大学, 脳研究所, 助教 (30646394)
Co-Investigator(Renkei-kenkyūsha) ONODERA Osamu  新潟大学, 脳研究所, 教授 (20303167)
Project Period (FY) 2014-04-01 – 2016-03-31
Keywords神経変性疾患 / 筋萎縮性側索硬化症 / TDP-43 / 病型分類 / 免疫組織化学
Outline of Final Research Achievements

A nuclear protein,TDP-43, is the major pathological protein in ALS. We attempted to clarify the corticalpathology in cases of sporadic amyotrophic lateral sclerosis (ALS: n = 96) using an antibody specific to phosphorylated TDP-43 (pTDP-43). The cases were divided into two groups: those without pTDP-43-positive neuronal cytoplasmic inclusions in the hippocampal dentate granule cells (classical ALS: ALS-C, n = 63), and those with such inclusions (ALS with temporal lesions: ALS-T, n = 33). Furthermore, the ALS-T cases were divided into two subgroups based on semi-quantitative estimation of pTDP-43-positive dystrophic neurites (DNs) in the temporal neocortex: ALS-T/few DNs (n = 22) and ALS-T/many DNs (n = 11). Considering the patient survival time and severity of motor neuron loss in each group, transition from ALS-C to ALS-T, or from ALS-T/few DNs to ALS-T/many DNs during the disease course appeared unlikely. Therefore, each of these three groups was regarded as an independent subtype.

Free Research Field

神経病理学

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Published: 2017-05-10  

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