OBJECTIVES: Type IV collagen (IV-C) abnormalities of skin and serum have been reported in patients with amyotrophic lateral sclerosis (ALS). However, there has been no study of urinary IV-C in ALS. The present study investigates urinary IV-C and the relation to its skin content in patients with ALS. MATERIAL AND METHODS: We studied IV-C immunoreactivity of skin and measured urinary levels of IV-C in ALS patients and controls. RESULTS: The basement membrane as well as blood vessels of skin in ALS patients was weakly positive for IV-C as compared with those of controls. Immunostaining became even weaker as ALS progressed. The urinary level of IV-C in ALS patients was significantly decreased as compared to diseased controls (P<0.001) and healthy controls (P<0.001), and was negatively and significantly associated with duration of symptoms (r=-0.85, P<0.001). There was an appreciable positive correlation between urinary IV-C levels and the density for IV-C immunoreactivity in ALS patients (r=0.84, P<0.01). CONCLUSION: These data suggest that a metabolic alteration of IV-C may occur in ALS patients and decreased levels of urinary IV-C may be related to the decreased IV-C immunoreactivity of skin in ALS.
OBJECTIVES: Type IV collagen (IV-C) abnormalities of skin and serum have been reported in patients with amyotrophic lateral sclerosis (ALS). However, there has been no study of urinary IV-C in ALS. The present study investigates urinary IV-C and the relation to its skin content in patients with ALS. MATERIAL AND METHODS: We studied IV-C immunoreactivity of skin and measured urinary levels of IV-C in ALSpatients and controls. RESULTS: The basement membrane as well as blood vessels of skin in ALSpatients was weakly positive for IV-C as compared with those of controls. Immunostaining became even weaker as ALS progressed. The urinary level of IV-C in ALSpatients was significantly decreased as compared to diseased controls (P<0.001) and healthy controls (P<0.001), and was negatively and significantly associated with duration of symptoms (r=-0.85, P<0.001). There was an appreciable positive correlation between urinary IV-C levels and the density for IV-C immunoreactivity in ALSpatients (r=0.84, P<0.01). CONCLUSION: These data suggest that a metabolic alteration of IV-C may occur in ALSpatients and decreased levels of urinary IV-C may be related to the decreased IV-C immunoreactivity of skin in ALS.
Authors: Nick S Verber; Stephanie R Shepheard; Matilde Sassani; Harry E McDonough; Sophie A Moore; James J P Alix; Iain D Wilkinson; Tom M Jenkins; Pamela J Shaw Journal: Front Neurol Date: 2019-04-03 Impact factor: 4.003
Authors: Stephanie R Shepheard; Tim Chataway; David W Schultz; Robert A Rush; Mary-Louise Rogers Journal: PLoS One Date: 2014-01-27 Impact factor: 3.240
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