Intrathymic pathogenesis of myasthenia gravis: transient expression of acetylcholine receptors on thymus-derived myogenic cells.

Differentiation of myogenic stem cells from undifferentiated thymic stem cells is thought to play a critical role in the pathogenesis of myasthenia gravis. The expression of membrane acetylcholine receptor (AChR) on the membranes of developing muscle clones in cultures of murine thymus reticulum was followed and found to be transient. AChR are first expressed shortly after fusion of myotubes. In subsequent stages of myogenic development, the density of homogenously distributed AChR is strongly increased, and, in addition, concentrated "hot spot" AChR areas appear. During further maturation, membrane AChR are lost. Highly mature myotubes (3 months in culture) lack substantial amounts of homogenous AChR, as well as hot spots.