Activation of Signal Transducer and Activator of Transcription-3 in the Spinal Cord of Sporadic Amyotrophic Lateral Sclerosis Patients

2009 ◽  
Vol 6 (3) ◽  
pp. 118-126 ◽  
Author(s):  
Noriyuki Shibata ◽  
Akiyoshi Kakita ◽  
Hitoshi Takahashi ◽  
Yuetsu Ihara ◽  
Keigo Nobukuni ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Amyotrophic Lateral Sclerosis ◽  
Sporadic Amyotrophic Lateral Sclerosis ◽  
Signal Transducer ◽  
Transcription 3 ◽  
Lateral Sclerosis

scholarly journals TDP-43 Vasculopathy in the Spinal Cord in Sporadic Amyotrophic Lateral Sclerosis (sALS) and Frontal Cortex in sALS/FTLD-TDP

2021 ◽  
Vol 80 (3) ◽  
pp. 229-239
Author(s):  
Isidro Ferrer ◽  
Pol Andrés-Benito ◽  
Margarita Carmona ◽  
Abdelilah Assialioui ◽  
Mónica Povedano
Keyword(s):  
Spinal Cord ◽  
Amyotrophic Lateral Sclerosis ◽  
Frontal Cortex ◽  
Blood Vessels ◽  
External Surface ◽  
Sporadic Amyotrophic Lateral Sclerosis ◽  
Double Labeling ◽  
Cortex Area ◽  
Altered Blood ◽  
Lateral Sclerosis

Abstract Sporadic amyotrophic lateral sclerosis (sALS) and FTLD-TDP are neurodegenerative diseases within the spectrum of TDP-43 proteinopathies. Since abnormal blood vessels and altered blood-brain barrier have been described in sALS, we wanted to know whether TDP-43 pathology also occurs in blood vessels in sALS/FTLD-TDP. TDP-43 deposits were identified in association with small blood vessels of the spinal cord in 7 of 14 cases of sALS and in small blood vessels of frontal cortex area 8 in 6 of 11 FTLD-TDP and sALS cases, one of them carrying a GRN mutation. This was achieved using single and double-labeling immunohistochemistry, and double-labeling immunofluorescence and confocal microscopy. In the sALS spinal cord, P-TDP43 Ser403-404 deposits were elongated and parallel to the lumen, whereas others were granular, seldom forming clusters. In the frontal cortex, the inclusions were granular, or elongated and parallel to the lumen, or forming small globules within or in the external surface of the blood vessel wall. Other deposits were localized in the perivascular space. The present findings are in line with previous observations of TDP-43 vasculopathy in a subset of FTLD-TDP cases and identify this pathology in the spinal cord and frontal cortex in a subset of cases within the sALS/FTLD-TDP spectrum.

Soluble iron accumulation induces microglial glutamate release in the spinal cord of sporadic amyotrophic lateral sclerosis

2019 ◽  
Vol 40 (2) ◽  
pp. 152-166
Author(s):  
Motoko Niida‐Kawaguchi ◽  
Akiyoshi Kakita ◽  
Noriko Noguchi ◽  
Miku Kazama ◽  
Kenta Masui ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Amyotrophic Lateral Sclerosis ◽  
Glutamate Release ◽  
Iron Accumulation ◽  
Sporadic Amyotrophic Lateral Sclerosis ◽  
Soluble Iron ◽  
Lateral Sclerosis
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