Lombardi G, Germain C, Uren J, Fiorillo M T, du Bois R M, Jones-Williams W, Saltini C, Sorrentino R, Lechler R
Department of Immunology, Imperial College School of Medicine, Hammersmith Hospital, London, United Kingdom.
J Immunol. 2001 Mar 1;166(5):3549-55. doi: 10.4049/jimmunol.166.5.3549.
Occupational exposure to small molecules, such as metals, is frequently associated with hypersensitivity reactions. Chronic beryllium (Be) disease (CBD) is a multisystem granulomatous disease that primarily affects the lung, and occurs in approximately 3% of individuals exposed to this element. Immunogenetic studies have demonstrated a strong association between CBD and possession of alleles of HLA-DP containing glutamic acid (Glu) at position 69 in the HLA-DP beta-chain. T cell clones were raised from three patients with CBD in whom exposure occurred 10 and 30 years previously. Of 25 Be-specific clones that were obtained, all were restricted by HLA-DP alleles with Glu at DP beta69. Furthermore, the proliferative responses of the clones were absolutely dependent upon DP beta Glu(69) in that a single amino acid substitution at this position abolished the response. As befits a disease whose pathogenesis involves a delayed type hypersensitivity response, the large majority of Be-specific clones secreted IFN-gamma (Th1) and little or no IL-4 (Th2) cytokines. This study provides insights into the molecular basis of DP2-associated susceptibility to CBD.
职业性接触小分子物质,如金属,常常与过敏反应相关。慢性铍(Be)病(CBD)是一种多系统肉芽肿性疾病,主要影响肺部,在接触该元素的个体中约有3%会发病。免疫遗传学研究表明,CBD与HLA - DPβ链第69位含有谷氨酸(Glu)的等位基因的携带密切相关。从三名分别在10年和30年前有过接触史的CBD患者中培养出T细胞克隆。在所获得的25个铍特异性克隆中,所有克隆均受DPβ69位为Glu的HLA - DP等位基因限制。此外,克隆的增殖反应绝对依赖于DPβGlu(69),因为该位置的单个氨基酸替换会消除反应。作为一种发病机制涉及迟发型超敏反应的疾病,绝大多数铍特异性克隆分泌干扰素 - γ(Th1),很少或不分泌白细胞介素 - 4(Th2)细胞因子。本研究为DP2相关的CBD易感性的分子基础提供了见解。