Age-related changes in BAFF and APRIL profiles and upregulation of BAFF and APRIL expression in patients with primary antibody deficiency.
Age-related changes in BAFF and APRIL profiles and upregulation of BAFF and APRIL expression in patients with primary antibody deficiency.
复制标题
DOI：
10.3892/ijmm.21.2.233
复制
发表时间：
2008-02
影响因子：
5.4
通讯作者：
R. Jin;H. Kaneko;Hiroko Suzuki;T. Arai;Takahide Teramoto;T. Fukao;N. Kondo
中科院分区：
文献类型：
--
作者：
R. Jin;H. Kaneko;Hiroko Suzuki;T. Arai;Takahide Teramoto;T. Fukao;N. Kondo
在一些常见的可变免疫缺陷(CVID)和免疫球蛋白(Ig)A缺陷(IGAD)患者中，已有报道肿瘤坏死因子(TNF)家族受体跨膜激活物和钙调节剂以及亲环素配体相互作用物(TACI)基因突变。来自TACI突变个体的B细胞对TACI配体a增殖诱导配体(APRIL)不产生免疫球蛋白和免疫球蛋白A，这可能表明同型转换受损。为阐明日本CVID和IGAD的发病机制，我们研究了CVID、IGAD和X连锁无丙种球蛋白血症(XLA)患者的肿瘤坏死因子家族成员TACI、APRIL、B细胞激活因子(BAFF)、B细胞成熟抗原(BCMA)和BAFF受体(BAFF-R)基因突变以及BAFF和APRIL的表达水平。我们还研究了年龄与血浆BAFF和APRIL水平的关系。本组患者未检测到致病基因突变。在健康受试者中，BAFF和APRIL血浆水平与年龄呈负相关。CVID、IGAD和XLA患儿的BAFF和APRIL水平均显著高于健康儿童。BAFF和APRIL表达水平升高可能部分反映了一抗缺陷的共同免疫学特征。
In some patients with common variable immunodeficiency (CVID) and immunoglobulin (Ig) A deficiency (IgAD), tumor necrosis factor (TNF) family receptor transmembrane activator and calcium-modulator and cyclophilin ligand interactor (TACI) gene mutations have been reported. B cells from individuals with TACI mutations do not produce IgG and IgA in response to the TACI ligand a proliferation-inducing ligand (APRIL) which probably suggests impaired isotype switching. To clarify the pathogenesis of CVID and IgAD of Japanese patients, we investigated the mutations of TNF family members TACI, APRIL, B-cell activating factor (BAFF), B-cell maturation antigen (BCMA) and BAFF receptor (BAFF-R) genes and the expression levels of BAFF and APRIL in patients with CVID, IgAD and X-linked agammaglobulinaemia (XLA). We also investigated the relationship between age and the blood plasma levels of BAFF and APRIL. The causative gene mutations of TNF family members in our patients were not detected. In healthy subjects, the BAFF and APRIL plasma levels correlated inversely with age. The BAFF and APRIL plasma levels of patients with CVID, IgAD and XLA were significantly higher than those of healthy children. Elevated BAFF and APRIL expression levels might partially reflect the common immunological feature of primary antibody deficiency.