Injection of anti-AChR antibodies in passive transfer experimental autoimmune myasthenia gravis (EAMG) results in increased degradation of acetylcholine receptor (AChR) and increased synthesis of AChR alpha-subunit mRNA. Passive transfer of anti-Main Immunogenic Region (MIR) mAb 35 in aged rats does not induce clinical signs of disease nor AChR loss. The expression of the AChR subunit genes was...

A 69-year-old Japanese female was admitted because of progressive nasal voice and dysphagia. Neurological examination revealed paresis of the soft palate with marked dysphagia and rhinolalia. Otherwise there was no weakness or easy fatigability in extraocular muscles and extremities. On laboratory test, anti-acetylcholine receptor antibody (anti-AChR Ab) was positive, while anti-muscle-specific...

Congenital myasthenic syndromes (CMSs) stem from genetic defects in endplate (EP)-specific presynaptic, synaptic, and postsynaptic proteins. The postsynaptic CMSs identified to date stem from a deficiency or kinetic abnormality of the acetylcholine receptor (AChR). All CMSs with a kinetic abnormality of AChR, as well as many CMSs with a deficiency of AChR, have been traced to mutations in AChR-...

Immunization of mammals with purified acetylcholine receptor protein (AChR) 1 results in an autoimmune response to skeletal muscle AChR which impairs neuromuscular transmission, causing experimental autoimmune myasthenia gravis (EAMG). In the introduction to the accompanying article (1) the striking similarities between EAMG and the human disease myasthenia gravis (MG) were reviewed. In that ar...

During neuromuscular synaptogenesis, neurally released agrin induces aggregation and tyrosine phosphorylation of acetylcholine receptors (AChRs) by acting through both the receptor tyrosine kinase MuSK (muscle-specific kinase) and the AChR-associated protein, rapsyn. To elucidate this signaling mechanism, we examined tyrosine phosphorylation of AChR-associated proteins, particularly addressing ...

The acetylcholine receptor (AChR) clusters of cultured rat myotubes contain two distinct, interdigitating, membrane domains, one enriched in AChR, the other poor in AChR but associated with sites of myotube-substrate contact (Bloch, R. J., and B. Geiger, 1980, Cell, 21:25-35). We have used two cholesterol-specific cytochemical probes, saponin and filipin, to investigate the lipid nature of thes...

Objetivo: Analisar as características da Miastenia Gravis (MG). Revisão bibliográfica: A MG é uma doença autoimune mediada por anticorpos que afeta a membrana pós-sináptica na junção neuromuscular, sendo produzida diferentes contra proteínas sináptica. Isso, geralmente, em mais de 85% dos casos causado um tipo anticorpo o receptor acetilcolina do músculo esquelético (AChR-ab). rara; incidência ...

Myasthenia gravis is an autoimmune disease characterized by circulating autoantibodies against the acetylcholine receptor (AChR) ~ present on the neuromuscular endplate. It is generally believed that these antibodies are responsible for an accelerated rate of AChR degradation, leading to muscular weakness and fatiguability (1). To date, treatment has involved either the administration of antich...

The acetylcholine receptor (AChR) clusters of cultured rat myotubes contain two distinct, interdigitating, membrane domains, one enriched in AChR, the other poor in AChR but associated with sites of myotube-substrate contact (Bloch, R.J., and B. Geiger, 1980, Cell, 21:25-35). We have used two cholesterol-specific cytochemical probes, saponin and filipin, to investigate the lipid nature of these...