Read e-book online Myasthenia Gravis and Related Disorders PDF

By Henry J. Kaminski

ISBN-10: 038772026X

ISBN-13: 9780387720265

ISBN-10: 0387720278

ISBN-13: 9780387720272

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Extra resources for Myasthenia Gravis and Related Disorders

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It can form heteromeric AChRs with α7. α9 can form heteromeric AChRs with α10 (54,55). Heteromeric neuronal AChRs are formed from α-subunits 2, 3, 4, or 6 in combination with β2 or β4 (1). α5 and β3 are present as additional subunits in AChRs in which ACh binding sites are formed by other α- and β-subunits 24 Lindstrom Fig. 4. Prominent acetylcholine receptor (AChR) subtypes. AChR subtypes are defined by their subunit compositions. The arrangement of AChR subunits around the channel is known for Torpedo electric organ muscle type AChRs (21) and is inferred for other types based on this example.

Among MG patients, the absolute concentration of antibodies to AChR does not correlate closely with severity (130,131), but, in general, patients with only ocular signs have lower concentrations than do patients with generalized MG, and changes in clinical state are usually paralleled by changes in antibody concentrations (132). The basic mechanisms by which autoantibodies to AChRs impair neuromuscular transmission appear to be very similar in MG and chronic EAMG (86,89). There are forms of both MG and EAMG that are passively transferred by autoantibodies to AChRs.

Twelve percent of MG patients have thymoma, and 35% of thymoma patients have MG (112). , titin (113–115). Thymic tumor cells do not usually express α1 AChRs (99), but traces of AChRs have been detected in thymus myoid cells, thymic epithelia, and dendritic cells (116,117). The autoantibodies to AChRs might result from a bystander adjuvant effect of the disruption caused by the tumor to an immune regulatory organ that contains traces of AChRs. Alternatively, viruses or other factors that cause the tumor might also cause disruption of AChR-expressing cells.

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Myasthenia Gravis and Related Disorders by Henry J. Kaminski


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