Anti-Sulfoglucuronosyl Paragloboside Antibody: A Potential Serologic Marker of Amyotrophic Lateral Sclerosis.
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by progressive degeneration of upper and lower motor neurons. Although the etiology of ALS is obscure, genetic studies of familiar ALS suggest a multifactorial etiology for this condition. Similarly, there probably are multiple causes for sporadic ALS. Autoimmune-mediated motor neuron dysfunction is one proposed etiology for sporadic ALS. In the present study, anti-glycolipid antibodies including GM1, GD1b, GD3, and sulfoglucuronosyl paragloboside (SGPG) were investigated in the sera of a large number of patient samples, including 113 ALS patients and 50 healthy controls, by means of enzyme-linked immunosorbent assay with affinity parametric complex criterion evaluation and thin-layer chromatography immunooverlay (immuno-TLC). Anti-SGPG antibodies were found in the sera of 13.3% ALS patients (15 out of 113). The highest titer reached 1:1600. The presence of anti-SGPG antibodies in the serum samples was also confirmed by immuno-TLC. Importantly, a multiple logistic regression analysis showed that the presence of anti-SGPG antibody was positively correlated with age (p < .01) and negatively correlated with ALS Functional Rating Scale score (p < .05). Moreover, the localization of SGPG-immunoreactivity on the motor neurons of rat spinal cord and a mouse motor neuronal cell line, NSC-34 was observed by an immunofluorescence method. These data suggest that SGPG could represent a specific pathogenic antigen in those ALS patients. The presence of anti-SGPG antibodies in the serum of ALS patients should represent a diagnostic biomarker of ALS, and it could reflect the severity of the disease.
Department of Neuroscience and Regenerative Medicine, Medical College of Georgia, Augusta University, GA, USA.
Dongpei
D
Li
Laboratory of Biomembrane and Biofunctional Chemistry, Faculty of Advanced Life Science, Frontier Research Center for Advanced Material and Life Science, Hokkaido University, Sapporo, Japan.
Seigo
S
Usuki
Department of Neurology, ALS Clinic, Medical College of Georgia, Augusta University, GA, USA.
Brandy
B
Quarles
Department of Neurology, ALS Clinic, Medical College of Georgia, Augusta University, GA, USA.
Michael H
MH
Rivner
Department of Neuroscience and Regenerative Medicine, Medical College of Georgia, Augusta University, GA, USA.
Toshio
T
Ariga
Department of Neuroscience and Regenerative Medicine, Medical College of Georgia, Augusta University, GA, USA Department of Neurology, ALS Clinic, Medical College of Georgia, Augusta University, GA, USA ryu@augusta.edu.
Robert K
RK
Yu
Functional Rating Scale; affinity parametric complex criterion; amyotrophic lateral sclerosis; anti-glycolipid antibody; ganglioside; sulfoglucuronosyl paragloboside
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