OBJECTIVES: 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. MATERIALS AND 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.
OBJECTIVES: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. MATERIALS AND 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 ALSpatients 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 ALSpatients. The optical density of TDP-43+ cells in the epidermis in ALSpatients 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 ALSpatients. 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.
Authors: Shu Yang; Katharine Y Zhang; Ruvini Kariawasam; Monique Bax; Jennifer A Fifita; Lezanne Ooi; Justin J Yerbury; Garth A Nicholson; Ian P Blair Journal: Neurotox Res Date: 2015-05-27 Impact factor: 3.911
Authors: D Michal Freedman; Rochelle E Curtis; Sarah E Daugherty; James J Goedert; Ralph W Kuncl; Margaret A Tucker Journal: Cancer Causes Control Date: 2012-10-23 Impact factor: 2.506
Authors: Kathryne Kirk; Chris Gennings; Jonathan C Hupf; Saba Tadesse; Marilena D'Aurelio; Hibiki Kawamata; Federica Valsecchi; Hiroshi Mitsumoto; Giovanni Manfredi Journal: Ann Neurol Date: 2014-08-21 Impact factor: 10.422