Agrin and LRP4 antibodies as new biomarkers of myasthenia gravis.

Abstract

Myasthenia gravis (MG) is a common disorder that affects the neuromuscular junction. It is caused by antibodies against acetylcholine receptor and muscle-specific tyrosine kinase; however, some MG patients do not have antibodies against either of the proteins. Recent studies have revealed antibodies against agrin and its receptor LRP4-both critical for neuromuscular junction formation and maintenance-in MG patients from various populations. Results from experimental autoimmune MG animal models indicate that anti-LRP4 antibodies are causal to MG. Clinical studies have begun to reveal the significance of the new biomarkers. With their identification, MG appears to be a complex disease entity that can be classified into different subtypes with different etiology, each with unique symptoms. Future systematic studies of large cohorts of well-diagnosed MG patients are needed to determine whether each subtype of patients would respond to different therapeutic strategies. Results should contribute to the goal of precision medicine for MG patients. Anti-agrin and anti-LRP4 antibodies are also detectable in some patients with amyotrophic lateral sclerosis or Lou Gehrig's disease; however, whether they are a cause or response to the disorder remains unclear.

• Authors: Min Yan, Guang-Lin Xing, Wen-Cheng Xiong, Lin Mei
• Journal: Annals of the New York Academy of Sciences (Ann N Y Acad Sci) Vol. 1413 Issue 1 Pg. 126-135 (02 2018) ISSN: 1749-6632 [Electronic] United States
• PMID: 29377176 (Publication Type: Journal Article, Research Support, N.I.H., Extramural, Research Support, Non-U.S. Gov't, Research Support, U.S. Gov't, Non-P.H.S., Review)
• Copyright: © 2018 New York Academy of Sciences.

Chemical References

• Agrin
• Autoantibodies
• LDL-Receptor Related Proteins
• LRP4 protein, human
• Receptors, Cholinergic
• MUSK protein, human
• Receptor Protein-Tyrosine Kinases

Topics

• Agrin (genetics, immunology)
• Animals
• Autoantibodies (immunology)
• Humans
• LDL-Receptor Related Proteins (genetics, immunology)
• Mice
• Myasthenia Gravis (immunology)
• Neuromuscular Junction (immunology, pathology)
• Receptor Protein-Tyrosine Kinases (genetics, immunology)
• Receptors, Cholinergic (genetics, immunology)