Abstract
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by specific loss of motor neurons in the spinal cord and brain stem. Currently, there are limited options for treating ALS and further investigation of the disease etiology and ALS disease progression need to be completed. There is an urgent need to identify biomarkers to detect and study disease progression in ALS. Glial fibrillary acidic protein (GFAP) is an intermediate filament protein that is expressed by a number of cells related to the central nervous system including glial cells and ependymal cells. Recent studies indicated that significant levels of GFAP protein were detected in peripheral tissues, such as skeletal muscle. In this study, we hypothesized that levels of GFAP in blood represent a biomarker of disease progression in ALS. To test this specific hypothesis, we used a rat model of familial ALS (SOD1G93A transgenic), which has been extensively used to understand the complexity of this devastating disease. Disease progression in a cohort of male and female SOD1G93A transgenic rats was monitored by motor function, and blood samples were collected when these animals reached disease end-stage. We measured GFAP protein levels by ELISA and found no correlation between GFAP concentration and disease progression in either serum and plasma samples of SOD1G93A transgenic. Further investigation would be required in order to implicate blood GFAP as a potential biomarker for ALS.
Highlights
Amyotrophic lateral sclerosis (ALS) is a debilitating neuromuscular disease caused by the loss of motor neurons in the brain and spinal cord [1, 2]
We recently reported that an overall increase of Glial fibrillary acidic protein (GFAP) expression following disease progression was determined in the limb muscle of a rat model of familial ALS (SOD1G93A transgenic) [24]
GFAP measurement by ELISA revealed that there was no significant difference in serum GFAP concentrations (Figure 1A)
Summary
Amyotrophic lateral sclerosis (ALS) is a debilitating neuromuscular disease caused by the loss of motor neurons in the brain and spinal cord [1, 2]. 90% of all ALS patients are considered as sporadic while the remaining 10% are caused by a familial/hereditary genetic etiology. There are a number of genes in which ALS-causing mutations have been characterized, such as SOD1, TARDPB, FUS, and C9ORF72 [2]. While the exact mechanism of pathology remains unknown, multiple pathologies have been involved in ALS; abnormal protein misfolding.
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