γ重链病一例新病例：临床、免疫化学及结构特征分析

A new case of gamma heavy chain disease: clinical, immunochemical and structural characterization.

作者信息

Carbonara A O, Paolino W, Malavasi F, Levis A, Stramignoni A, de Lange G, van Loghem E, Franklin E C

出版信息

Vox Sang. 1979;37(2):89-95. doi: 10.1111/j.1423-0410.1979.tb02276.x.

DOI:10.1111/j.1423-0410.1979.tb02276.x

Abstract

A new case of gamma heavy chain disease (gamma HCD) is described in a 77-year-old woman. The serum and urine contain an M-component and electrophoretic, antigenic and ultracentrifugal properties resembling those of the Fc fragment of IgG globulin. Analysis on SDS-polyacrylamide gel electrophoresis and ultracentrifugal studies show that gamma HCD portein is present in the serum as a dimer with a molecular weight of 58,000 daltons. Analysis of isotypic and allotypic markers along with the structural studies show that this HCD protein belongs to the IgG subclass and that deletion includes the total VH and CH1 regions with sequence starting at residue 225 in the middle of the hinge region.

摘要

本文描述了一例发生在77岁女性身上的γ重链病（gamma HCD）。患者血清和尿液中含有一种M成分，其电泳、抗原及超速离心特性与IgG球蛋白的Fc片段相似。SDS-聚丙烯酰胺凝胶电泳分析和超速离心研究表明，血清中的γHCD蛋白以分子量为58,000道尔顿的二聚体形式存在。对同种型和异型标记的分析以及结构研究表明，这种HCD蛋白属于IgG亚类，缺失部分包括整个VH和CH1区域，序列起始于铰链区中部的第225位残基。