Titin and ryanodine receptor antibodies in myasthenia gravis

Acta Neurologica Scandinavica. Supplementum
Geir Olve SkeieNils Erik Gilhus

Abstract

Some myasthenia gravis (MG) patients have antibodies against skeletal muscle antigens in addition to the acetylcholine receptor (AChR). Two major antigens for non-AchR antibodies in MG are the Ca(2+) release channel of the sarcoplasmic reticulum, the ryanodine receptor (RyR) and titin, a gigantic filamentous muscle protein essential for muscle structure, function and development. RyR and titin antibodies are found mainly in thymoma MG patients and in a few late-onset MG patients and correlate with a severe MG disease. The presence of titin antibodies, which bind to key regions near the A/I junction and in the central I-band, correlates with myopathy. The immunosuppressant (FK506), which enhances Ca(2+) release from the RyR, seems to have a symptomatic effect on MG patients with RyR antibodies. The RyR antibodies recognize a region near the N-terminus important for channel regulation and inhibit Ca(2+) release in vitro. However, evidence that antibodies against the intracellular antigens RyR and titin are pathogenic in vivo is still missing.

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Citations

Jan 22, 2009·Journal of Neurology·Jone Furlund OweNils Erik Gilhus
Jan 27, 2010·Neuroscience Bulletin·Sha Huang, Li-Ming Tan
Jun 16, 2014·Computational Biology and Chemistry·Tapati SarkarAshesh Nandy
Dec 18, 2015·Expert Review of Clinical Immunology·Amelia EvoliEmanuela Bartoccioni
Mar 11, 2009·Expert Review of Neurotherapeutics·Nils Erik Gilhus
Mar 21, 2009·Journal of Neuroimmunology·Espen Homleid AlsethGeir Olve Skeie
Feb 4, 2009·Journal of Neuroimmunology·Takahiro MarutaMasahito Yamada
Oct 9, 2007·Clinical Radiology·G-C RutherfordA O'Connor
May 5, 2010·Therapeutic Apheresis and Dialysis : Official Peer-reviewed Journal of the International Society for Apheresis, the Japanese Society for Apheresis, the Japanese Society for Dialysis Therapy·Jun-Feng LiuYong Gu
May 13, 2014·Journal of Clinical Neuroscience : Official Journal of the Neurosurgical Society of Australasia·Lei ZhangChuanzheng Lv
Aug 23, 2016·Annals of Clinical and Translational Neurology·Jae-Yun LeeKevin C O'Connor
Jul 18, 2016·Journal of the Neurological Sciences·W GrisoldW N Löscher
Feb 28, 2018·Frontiers in Immunology·Inga Koneczny
Sep 19, 2014·The Journal of Immunology : Official Journal of the American Association of Immunologists·Anette S B WolffEystein S Husebye
Feb 26, 2016·Journal of Clinical Neuromuscular Disease·Pamela McCombeStefan Blum
Dec 18, 2020·Frontiers in Neurology·Konstantinos Lazaridis, Socrates J Tzartos
May 28, 2021·Expert Review of Clinical Immunology·Amelia EvoliValentina Damato
Dec 19, 2021·Neurology and Therapy·Andreea DragusinCristina Tiu

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