Stability of mutant superoxide dismutase-1 associated with familial amyotrophic lateral sclerosis determines the manner of copper release and induction of thioredoxin in erythrocytes

Biochemical and Biophysical Research Communications
Y OgawaS Sakoda

Abstract

We analyzed mutant superoxide dismutase-1 (SOD-1) in erythrocytes from patients with familial amyotrophic lateral sclerosis (FALS) by using ion exchange chromatography and HPLC/electrospray ionization mass spectrometry and were able to divide mutant SOD-1 proteins into a stable form including G37R and H46R, and an unstable form including I149T and a two base pair deletion mutant. Each mutant sample showed abnormal copper peaks in different chromatographic fractions without relation to SOD-1 activities. In addition, thioredoxin, known as an antioxidant molecule, was markedly increased in the stable form but not in the unstable form. These results suggest the presence of different pathways leading to motor neuron death between stable and unstable mutants.

Citations

Apr 17, 2001·Neuropathology : Official Journal of the Japanese Society of Neuropathology·Y WatanabeK Nakashima
Jun 15, 2006·Antioxidants & Redox Signaling·Yoshiaki Furukawa, Thomas V O'Halloran
Apr 12, 2014·Oxidative Medicine and Cellular Longevity·Daniela Silva-AdayaJorge Guevara
Mar 28, 2013·Oxidative Medicine and Cellular Longevity·Sonam ParakhJulie D Atkin
Sep 29, 2009·Clinical Nutrition : Official Journal of the European Society of Parenteral and Enteral Nutrition·Barkha P Patel, Mazen J Hamadeh
Jun 24, 2004·Brain Research. Brain Research Reviews·Andrea Malaspina, Jacqueline de Belleroche
Apr 21, 2005·Protein Science : a Publication of the Protein Society·Svetlana AntonyukS Samar Hasnain
Nov 30, 2002·The Journal of Biological Chemistry·Ashutosh Tiwari, Lawrence J Hayward
Apr 25, 2003·Amyotrophic Lateral Sclerosis and Other Motor Neuron Disorders : Official Publication of the World Federation of Neurology, Research Group on Motor Neuron Diseases·Kimiko InoueSaburo Sakoda
Sep 12, 2006·Amyotrophic Lateral Sclerosis : Official Publication of the World Federation of Neurology Research Group on Motor Neuron Diseases·Gardian C Y FongS L Ho
Nov 22, 2008·Biochemical Society Transactions·Willianne I M Vonk, Leo W J Klomp

❮ Previous
Next ❯

Related Concepts

Related Feeds

ALS: Genetics

Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder characterized by muscle weakness. Here is the latest research investigating genetic alterations in this genetically heterogeneous disorder.

ALS: Genetics

Amyotrophic Lateral Sclerosis (ALS) is a progressive neurodegenerative disorder characterized by muscle weakness. ALS is a genetically heterogeneous disorder with several causative genes. Here are the latest discoveries pertaining to the genetics of this disease.

Amyloid Lateral Sclerosis

Amyotrophic Lateral Sclerosis (ALS) is a progressive nervous system disease associated with the death of neurons that control voluntary muscles. Discover the latest research on ALS here.