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Can I read Roles of collagen Q in MuSK antibody-positive myasthenia gravis on EtoBox?
Roles of collagen Q in MuSK antibody-positive myasthenia gravis by Kinji Ohno; Kenji Otsuka; Mikako Ito is a Biochemistry, Genetics and Molecular Biology article available to read on EtoBox.
What is Roles of collagen Q in MuSK antibody-positive myasthenia gravis about?
The low-density lipoprotein receptor-related protein 4 (LRP4) and the muscle-specific receptor tyrosine kinase (MuSK) form a tetrameric protein complex on the postsynaptic membrane at the neuromuscular junction (NMJ). Binding of agrin to LRP4 triggers phosphorylation of MuSK. Activated MuSK drives clustering of acetylcholine receptor (AChR). Wnt ligands also directly bind to MuSK to induce AChR clustering. MuSK anchors the acetylcholinesterase (AChE)/collagen Q (ColQ) complex to the synaptic basal lamina. In addition, an extracellular proteoglycan, biglycan, binds to MuSK. Anti-MuSK autoantibodies (MuSK-IgG) are observed in 5-15% of autoimmune myasthenia gravis (MG) patients. MuSK-IgG blocks both ColQ-MuSK and LRP4-MuSK interactions. MuSK-IgG, LRP4, ColQ, and biglycan bind to the immunoglobulin-like domains 1 and 4 of MuSK. Lack of the effects of cholinesterase inhibitors in MuSK-MG patients is likely due to hindrance of ColQ-MuSK interaction by MuSK-IgG and subsequent deficiency of AChE observed in model mice, which, however, has not been proven in MuSK-MG patients. As ColQ enhances expression of membrane-bound MuSK, inhibition of ColQ-MuSK interaction by MuSK-IgG may account for
Who reads Roles of collagen Q in MuSK antibody-positive myasthenia gravis?
It is typically read by researchers, students, and practitioners in Biochemistry, Genetics and Molecular Biology.
- Author
- Kinji Ohno; Kenji Otsuka; Mikako Ito
- Publisher
- Elsevier BV
- Published
- 2016
- Language
- EN
- Field
- Biochemistry, Genetics and Molecular Biology (Life Sciences)