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Antibody Transfection into Neurons as a Tool to Study Disease Pathogenesis
Published on: September 26, 2012
Peripherin-IgG association with neurologic and endocrine autoimmunity
Jayne L Chamberlain1, Sean J Pittock, Anna-Maria Oprescu
1Department of Immunology, Mayo Clinic College of Medicine, Rochester, MN 55905, USA.
Journal of Autoimmunity
|January 12, 2010
Summary
Peripherin-IgG autoantibodies, previously seen in mice, are now identified in humans with neurological and endocrine conditions. This discovery links peripherin-IgG to autoimmune diseases affecting the nervous system and endocrine glands.
Area of Science:
- Neuroimmunology
- Endocrinology
- Autoimmunity
Background:
- Peripherin-IgG autoantibodies are known in non-obese type 1 diabetic (NOD) mice.
- Their role in human disease was previously unrecognized.
Purpose of the Study:
- To investigate the presence and clinical significance of peripherin-IgG autoantibodies in human patients.
- To identify the target antigen of these autoantibodies.
Main Methods:
- Blinded serum evaluation for neurological autoimmunity markers.
- Immunohistochemical and Western blot analysis to identify the target antigen.
- Clinical history review of seropositive patients.
Main Results:
- Identified 26 patients with IgG binding to neural elements; the target antigen was peripherin.
- Seropositive patients frequently exhibited dysautonomia (54%), neuropathies (30%), and endocrinopathies (35%).
- 73% of seropositive patients had autonomic dysfunction or endocrinopathy; 33% of patients with small fiber/autonomic neuropathies were seropositive.
Conclusions:
- Peripherin-IgG autoantibodies are associated with human neurological and endocrine autoimmune disorders.
- Neuronal elements targeted by peripherin-IgG may be an initial site for immune attack in endocrine autoimmunity.
- Further research is needed to determine if peripherin-IgG predicts small fiber neuropathy development.
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