Dr. Duygu Aydemir
Department of Medical Biochemistry, School of Medicine, Koc University, Istanbul, Turkey
Title: Edavarone treatment for the SOD1-mutated patients: It is a new hope for early onset ALS disease
Biography
Biography: Dr. Duygu Aydemir
Abstract
Amyotrophic lateral sclerosis (ALS) is described as a progressive and lethal disease characterized by the degeneration of motor neurons localized in brain, brain stem, and spinal cord. Several mechanisms including mitochondrial dysfunction, apoptosis, inflammation, protein degradation and oxidative stress are associated with the initiation of the neurodegenerative process of ALS. The free radical scavenger Edavarone was reported to reduce speed of disease progression in early phase ALS, however there are no published data indicating biochemical or neurophysiological markers to quantify the impact of Edavarone. Blood and serum samples were collected from three cousins with heterozygous Leu144Phe mutation on fifth exon of SOD-1 gene were administered with Edavarone for one year as after-before treatment periods. Afterwards oxidative stress metabolism was investigated via glucose-6 phosphate dehydrogenase (G6PD), 6-phosphoglucanate dehydrogenase (6PGD), glutathione reductase (GR), glutathione-s transferase (GST), glutathione peroxidase (GPx), TAS, TOS, total thiol and native thiol in the erythrocytes. Moreover, 28 trace element and mineral were investigated via ICP-MS in the erythrocytes and serum samples. Needle EMG, sensory nerve action potentials (SNAPs), demographics and neurological examination were used to evaluate neurological markers. First time in the literature, our data showed that Edavarone ameliorates biochemical and neurological biomarkers at the early phase.