TDP-43 levels are higher in platelets from patients with sporadic amyotrophic lateral sclerosis than in healthy controls.

Abstract:

:TAR DNA-binding protein 43 (TDP-43) is a major pathological protein of ubiquitinated inclusions in motor neurons of sporadic amyotrophic lateral sclerosis (ALS). TDP-43 is ubiquitously expressed and the majority of TDP-43 is normally localized to the nucleus. In motor neurons of patients with ALS, TDP-43 is not localized in the nucleus, relocates to the cytoplasm, and accumulates as cytoplasmic inclusions. Based on recent reports that TDP-43 is increased in the cytoplasmic fraction of peripheral blood mononuclear cells in sporadic ALS, and several studies on platelet dysfunction in ALS patients, we investigated the TDP-43 levels in platelets from patients with sporadic ALS. We measured TDP-43 levels with a sandwich enzyme-linked immunosorbent assay in platelets separated from whole blood, and compared the TDP-43 level in platelets from sporadic ALS (n = 19) patients with platelets from non-ALS controls (n = 21). The TDP-43 concentration in platelets was significantly higher in patients with ALS compared to age-matched controls. According to sub-analysis, the TDP-43 concentration in platelets tended to increase in ALS patients with longer disease duration, as well as with lower score on the ALS Functional Rating Scale Revised (ALSFRS-R), though the differences were not statistically significant. These results suggest that ALS also affects platelets in addition to motor neurons.

journal_name

Neurochem Int

authors

Hishizawa M,Yamashita H,Akizuki M,Urushitani M,Takahashi R

doi

10.1016/j.neuint.2018.12.009

subject

Has Abstract

pub_date

2019-03-01 00:00:00

pages

41-45

eissn

0197-0186

issn

1872-9754

pii

S0197-0186(18)30517-5

journal_volume

124

pub_type

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