寻常型天疱疮抗体与患者健康亲属主要组织相容性复合体的关联。
Linkage of pemphigus vulgaris antibody to the major histocompatibility complex in healthy relatives of patients.
作者信息
Ahmed A R, Mohimen A, Yunis E J, Mirza N M, Kumar V, Beutner E H, Alper C A
机构信息
Center for Blood Research, Harvard School of Dental Medicine, Boston, Massachusetts.
出版信息
J Exp Med. 1993 Feb 1;177(2):419-24. doi: 10.1084/jem.177.2.419.
Abstract
Pemphigus vulgaris (PV) is an autoimmune disease caused by high concentrations of antibody to an epidermal cadherin. The disease is associated with two kinds of HLA-DR4, DQ8 haplotypes dominantly distributed among Jewish patients, and these plus DR6, DQ5 haplotypes in non-Jewish patients. Low levels of the PV antibody were found in 48% of a total of 120 asymptomatic parents, children, and siblings of 31 patients, thus exhibiting dominant inheritance. The inheritance of these low levels of antibody in asymptomatic relatives was linked to the major histocompatibility complex with a highly significant logarithm of the odds score of 9.07, almost always to a DR4 or DR6 haplotype of the patient. Disease appears to occur in susceptible individuals with low levels of antibody when a second factor, either environmental or genetic, induces high levels, sufficient to produce blisters.
摘要
寻常型天疱疮（PV）是一种由针对表皮钙黏蛋白的高浓度抗体引起的自身免疫性疾病。该疾病与两种主要分布在犹太患者中的HLA - DR4、DQ8单倍型相关，以及非犹太患者中的这些单倍型加上DR6、DQ5单倍型。在31例患者的120名无症状父母、子女和兄弟姐妹中，48%发现了低水平的PV抗体，从而表现出显性遗传。无症状亲属中这些低水平抗体的遗传与主要组织相容性复合体相关，优势对数得分高达9.07，具有高度显著性，几乎总是与患者的DR4或DR6单倍型相关。当第二个因素，无论是环境因素还是遗传因素，诱导产生足以导致水疱的高水平抗体时，疾病似乎会在抗体水平较低的易感个体中发生。
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Linkage of pemphigus vulgaris antibody to the major histocompatibility complex in healthy relatives of patients.寻常型天疱疮抗体与患者健康亲属主要组织相容性复合体的关联。
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