Familial amyotrophic lateral sclerosis: a SOD1-unrelated Japanese family of bulbar type with Bunina bodies and ubiquitin-positive skein-like inclusions in lower motor neurons

2006 ◽  
Vol 113 (2) ◽  
pp. 205-211 ◽  
Author(s):  
Asako Tagawa ◽  
Chun-Feng Tan ◽  
Koki Kikugawa ◽  
Masayuki Fukase ◽  
Ryoichi Nakano ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Motor Neurons ◽  
Familial Amyotrophic Lateral Sclerosis ◽  
Japanese Family ◽  
Bunina Bodies ◽  
Lateral Sclerosis

scholarly journals Autophagy Is a Common Degradation Pathway for Bunina Bodies and TDP-43 Inclusions in Amyotrophic Lateral Sclerosis

2019 ◽  
Vol 78 (10) ◽  
pp. 910-921 ◽  
Author(s):  
Fumiaki Mori ◽  
Yasuo Miki ◽  
Tomoya Kon ◽  
Kunikazu Tanji ◽  
Koichi Wakabayashi
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Motor Neurons ◽  
Cathepsin B ◽  
Cathepsin D ◽  
Early Stage ◽  
Degradation Pathway ◽  
Sporadic Amyotrophic Lateral Sclerosis ◽  
Bunina Bodies ◽  
Related Proteins ◽  
Lateral Sclerosis

Abstract Bunina bodies (BBs) coexisting with TDP-43-immunoreactive (TDP-43-IR) skein-like inclusions (SIs) and round inclusions (RIs) in lower motor neurons are a frequent feature of sporadic amyotrophic lateral sclerosis (sALS). Since previous studies have shown that BBs and TDP-43-IR inclusions are often detected in association with autophagy-related structures (autophagosomes and autolysosomes), we examined the anterior horn cells (AHCs) of the spinal cord from 15 patients with sALS and 6 control subjects, using antibodies against autophagy-related proteins (LC3, cathepsin B, and cathepsin D). Among AHCs with SIs, 43.9% contained BBs, whereas 51.7% of AHCs with RIs did so. The cytoplasm of AHCs showed diffuse immunoreactivity for LC3, cathepsin B and cathepsin D in both sALS and controls. Ultrastructurally, SIs and mature BBs contained autophagosomes and autolysosomes. Mature BBs were localized in the vicinity of SIs. RIs also contained autophagosomes, autolysosomes, and early-stage BBs. These findings suggest that autophagy is a common degradation pathway for BBs and TDP-43-IR inclusions, which may explain their frequent coexistence.

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