Increased expression of TDP-43 in the skin of amyotrophic lateral sclerosis.
OBJECTIVE
Transactivation-responsive DNA-binding protein-43 (TDP-43) was identified as a major component of the ubiquitin-positive inclusions in sporadic amyotrophic lateral sclerosis (ALS). However, there has been no study of TDP-43 in ALS skin. The present study investigates TDP-43 in ALS skin.


METHODS
We made a quantitative immunohistochemical study of the expression of TDP-43 in the skin from 15 patients with ALS and 15 control subjects.


RESULTS
The proportion of TDP-43-positive (TDP-43+) cells in the epidermis in ALS patients was significantly higher (P < 0.001) than in controls. There was a significant positive relationship (r = 0.62, P < 0.02) between the proportion and duration of illness in ALS patients. The optical density of TDP-43+ cells in the epidermis in ALS patients is markedly stronger (P < 0.001) than in controls. There was a significant positive relation (r = 0.72, P < 0.01) between the immunoreactivity and duration of illness in ALS patients.


CONCLUSIONS
These data suggest that changes of TDP-43 in ALS skin are likely to be related to the disease process and that metabolic alterations of TDP-43 may take place in the skin of patients with ALS.
Suzuki
M
M
Department of Neurology, Teikyo University Chiba Medical Center, Ichihara, Japan.
Mikami
H
H
Watanabe
T
T
Yamano
T
T
Yamazaki
T
T
Nomura
M
M
Yasui
K
K
Ishikawa
H
H
Ono
S
S
D000368:Aged; D000690:Amyotrophic Lateral Sclerosis / Q000473:pathology*; D002452:Cell Count / Q000379:methods; D004268:DNA-Binding Proteins / Q000378:metabolism*; D004817:Epidermis / Q000473:pathology*; D005260:Female; D005786:Gene Expression Regulation / Q000502:physiology*; D006801:Humans; D008297:Male; D008875:Middle Aged; D011897:Random Allocation; D013223:Statistics as Topic
D004268:DNA-Binding Proteins