MYASTHENIA-GRAVIS - RECOGNITION OF A HUMAN AUTOANTIGEN AT THE MOLECULAR-LEVEL

The symptoms of myasthenia gravis are primarily or exclusively due to an autoimmune response against the muscle nicotinic acetylcholine rece ptor (AChR) and this has been the object of intensive investigations f or almost 20 years. A detailed picture at the molecular level of the i nteraction of this autoantigen with the key elements involved in the a utoimmune response, such as anti-AChR antibodies, the T-cell receptor and restricting major histocompatibility complex molecules, is now eme rging for both human myasthenia gravis and its experimental model, exp erimental autoimmune myasthenia gravis. Here, Maria Pia Protti and col leagues focus on the molecular interactions occurring in human myasthe nia gravis and summarize recent information on pathogenic mechanisms o f the autoimmune response, and the structure of epitopes recognized by B cells and CD4+ T cells of myasthenic patients on the AChR molecule.