Contribution of cellular immune dysregulation to myasthenia gravis pathology [0.03%]
细胞免疫调节障碍在重症肌无力病理中的作用
Alexandra C Bayer,Richard J Nowak,Kevin C OConnor
Alexandra C Bayer
Myasthenia gravis (MG) is an autoimmune disorder in which autoantibodies attack proteins at the neuromuscular junction, resulting in impaired neuromuscular transmission. Like other autoimmune diseases, MG arises when the immune system fails...
Sally Spendiff,Hanns Lochmüller,Ricardo A Maselli
Sally Spendiff
Congenital myasthenic syndromes (CMS) result from impaired neuromuscular transmission and are due to genetic mutations in one of several genes involved in the development, function, or maintenance of the neuromuscular junction (NMJ). The cl...
Shadi El-Wahsh,Stephen Reddel
Shadi El-Wahsh
Lambert Eaton Myasthenic Syndrome (LEMS) is a pre-synaptic neuromuscular junction disorder characterised clinically by leg-predominant proximal weakness with spread of weakness distally and cranially with increasing severity as well as redu...
Saiju Jacob,James F Howard Jr
Saiju Jacob
Nearly 90 % of generalized myasthenia gravis (MG) patients have IgG1 or IgG3 antibodies against the acetylcholine receptor (AChR). Acetylcholine receptor antibodies induce neuromuscular transmission defect by various potential mechanisms in...
Amol K Bhandage,Jiaxin Chen,Henry J Kaminski et al.
Amol K Bhandage et al.
Biomarkers are measurable indicators to assess physiological processes, disease states, or therapy responses. In myasthenia gravis (MG), biomarkers are critical for diagnosis, monitoring, and treatment optimization. Despite advances in MG d...
Special populations in myasthenia gravis: Early, late, and very late-onset MG [0.03%]
重症肌无力的特殊人群:早发、迟发和极迟发的MG患者
Sarah Hoffmann
Sarah Hoffmann
The age at disease onset significantly influences the clinical course, pathophysiology, and treatment response in Myasthenia Gravis (MG). This chapter examines how immunological profiles, autoantibody prevalence, thymic pathology, genetic a...
Nils Erik Gilhus,Ari Breiner
Nils Erik Gilhus
MG epidemiology may provide clues to disease etiology and pathogenesis. It is crucial for health planning and to estimate societal needs for health care resources. Recent high-quality studies have found an annual MG incidence of approximate...
Patricia M Sikorski,Linda L Kusner
Patricia M Sikorski
Animal models offer a platform to advance our understanding of myasthenia gravis (MG), an autoimmune disorder characterized by impaired neuromuscular transmission. Experimental autoimmune MG models (EAMG) actively induce autoimmunity throug...
Yu-Fang Huang,Robyn L K Verpalen,Anna Rostedt Punga et al.
Yu-Fang Huang et al.
Understanding the pathophysiology of Myasthenia Gravis (MG) and developing effective treatments requires using cell models that replicate key features of the disease, particularly those involved in the autoimmune response and neuromuscular ...
Structure and function of the neuromuscular junction in health and myasthenia gravis [0.03%]
健康和重症肌无力的神经肌肉接头的结构与功能
Jaap J Plomp,Richard G Webster
Jaap J Plomp
The neuromuscular junction (NMJ) connects a motor neuron to a skeletal muscle cell. Cholinergic synaptic transmission enables muscle contraction, which is crucial for survival. Although synaptic communication at the NMJ is robust, with an i...