Tomomi Tsukie1, Hiroaki Masaki1, Sohei Yoshida2, Mikio Fujikura1, Seiitsu Ono1. 1. Department of Neurology, Teikyo University Chiba Medical Center, 3426-3 Anesaki, Ichihara, Chiba 299-0111, Japan. 2. Department of Health Sciences, Kansai University of Health Sciences, 2-11-1 Wakaba, Kumatori-cho, Sennan-gun, Osaka 590-0482, Japan.
Abstract
PURPOSE: The Kii Peninsula of Japan, together with Guam and West New Guines, has one of the highest incidences of amyotrophic lateral sclerosis (Kii ALS) in the world. There is a controversy whether the etiology is the same or not between sporadic ALS and Kii ALS. Skin studies from patients with sporadic ALS have shown unique pathological and biochemical abnormalities. However, there has been no report of collagen content of the skin Kii ALS patients. METHODS: The skin tissues from Kii ALS patients were studied by electron microscopy and their collagen contents were examined. RESULTS: On electron microscopy the most conspicuous finding in Ki ALS was the smaller diameter of collagen fibrils. The collagen content per dry weight (mg) of the samples in Kii LAS was significantly decreased (P<0.001) than in controls. In Kii ALS patients the more severely affected pathological samples showed the greater decrease. In addition, there was a significant negative correlation (r = -0.88, P<0.01) between the collagen and duration of illness in the Kii ALS patients, but there was no such correlation in controls. CONCLUSION; These results indicate that the metabolism of skin collagen might be affected in the disease process of Kii ALS.
PURPOSE: The Kii Peninsula of Japan, together with Guam and West New Guines, has one of the highest incidences of amyotrophic lateral sclerosis (Kii ALS) in the world. There is a controversy whether the etiology is the same or not between sporadic ALS and Kii ALS. Skin studies from patients with sporadic ALS have shown unique pathological and biochemical abnormalities. However, there has been no report of collagen content of the skin Kii ALS patients. METHODS: The skin tissues from Kii ALS patients were studied by electron microscopy and their collagen contents were examined. RESULTS: On electron microscopy the most conspicuous finding in Ki ALS was the smaller diameter of collagen fibrils. The collagen content per dry weight (mg) of the samples in Kii LAS was significantly decreased (P<0.001) than in controls. In Kii ALS patients the more severely affected pathological samples showed the greater decrease. In addition, there was a significant negative correlation (r = -0.88, P<0.01) between the collagen and duration of illness in the Kii ALS patients, but there was no such correlation in controls. CONCLUSION; These results indicate that the metabolism of skin collagen might be affected in the disease process of Kii ALS.
Authors: Csaba Konrad; Hibiki Kawamata; Kirsten G Bredvik; Andrea J Arreguin; Steven A Cajamarca; Jonathan C Hupf; John M Ravits; Timothy M Miller; Nicholas J Maragakis; Chadwick M Hales; Jonathan D Glass; Steven Gross; Hiroshi Mitsumoto; Giovanni Manfredi Journal: Mol Neurodegener Date: 2017-10-24 Impact factor: 14.195
Authors: Manuel Chiusa; Wen Hu; Jozef Zienkiewicz; Xiwu Chen; Ming-Zhi Zhang; Raymond C Harris; Roberto M Vanacore; Jennifer A Bentz; Giuseppe Remuzzi; Ariela Benigni; Agnes B Fogo; Wentian Luo; Stavroula Mili; Matthew H Wilson; Roy Zent; Jacek Hawiger; Ambra Pozzi Journal: J Cell Biol Date: 2020-09-07 Impact factor: 10.539