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Heavy chain disease is a form of paraproteinemia with a proliferation of cells producing immunoglobulin heavy chains.〔(【引用サイトリンク】title=Heavy Chain Diseases: Plasma Cell Disorders: Merck Manual Home Edition )〕 This disease is characterized by an excessive production of heavy chains that are short and truncated. These heavy chain disease proteins have various deletions, mainly in their amino-terminal part, which causes the heavy chains to lose the ability to form disulfide bonds with the light chains. The defect in the immunoglobulins presumably arises during somatic hypermutation. Deletion of the N-terminal part of the heavy chain disease protein leads to aggregation and signaling of the B cell receptor, presumably due to the loss of the anti-aggregating properties of the light chain. ==Classification== There are four forms: * alpha chain disease (Seligmann's disease) * gamma chain disease (Franklin's disease) * mu chain disease * delta chain disease 抄文引用元・出典: フリー百科事典『 ウィキペディア(Wikipedia)』 ■ウィキペディアで「Heavy chain disease」の詳細全文を読む スポンサード リンク
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