The mechanism of antibody production using the model of primary immunodeficiency, especially IgA deficiency

使用原发性免疫缺陷，特别是 IgA 缺陷模型研究抗体产生机制

• 批准号: 18591188
• 负责人: KANEKO Hideo
• 金额: $ 2.51万
• 依托单位: Gifu University
• 依托单位国家: 日本
• 项目类别: Grant-in-Aid for Scientific Research (C)
• 财政年份: 2006
• 资助国家: 日本
• 起止时间: 2006 至 2007
• 项目状态: 已结题
• 来源: https://kaken.nii.ac.jp/grant/KAKENHI-PROJECT-18591188/
• 关键词: CVI; IgA deficiency; TNF family; TNFR family; IgA subclass; APRIL; BAFF; lgA損症; lgAサブクラス

IgA deficiency (IgAD) is the most common immunodeficiency, but the pathogenesis of most cases of IgAD is poorly understood. The gene and protein expression levels of members of the IgA subclasses in IgAD patients were analyzed by a reverse transcriptase (RT) -PCR method that could differentiate between α 1 and α 2 gene expression. Three selective, 5 partial and 2 secondary IgAD patients were examined. Peripheral blood mononuclear cells that were unstimulated or stimulated with TGF- β 1 and PMA for 24 hours were cultured. The IgAl/IgA2 expression ratios were measured by zone densitometry. Three bands appeared (the α 1 and α2 genes, and a hetero-duplex formation), owing to the difference of 39 bases between α 1 and α2 mRNAs. In controls, there were no significant differences in the IgAl/IgA2 ratios between unstimulated and stimulated cells. In selective IgAD patients, both α1 and α2 gene expression were induced following stimulation, and α1 gene expression was induced more dominantly than in the other IgAD patients following stimulation. Based on our results, it was thought that suppression of α 1 gene expression may be related to the pathogenesis of IgAD.

IgA缺乏症（IgAD）是最常见的免疫缺陷，但大多数病例的发病机制尚不清楚。采用区分α 1和α 2基因表达的逆转录酶(RT) -PCR方法分析IgAD患者中IgA亚类成员的基因和蛋白表达水平。选择性IgAD 3例，部分IgAD 5例，继发性IgAD 2例。培养未刺激或TGF- β 1和PMA刺激24小时的外周血单个核细胞。区密度法测定IgAl/IgA2表达比。由于α 1和α2 mrna之间存在39个碱基的差异，出现了α 1和α2基因的3条条带，并形成异双工结构。在对照组中，未受刺激和受刺激细胞之间的IgAl/IgA2比值没有显著差异。在选择性IgAD患者中，α1和α2基因在刺激后均被诱导表达，且α1基因在刺激后的表达较其他IgAD患者更为明显。基于我们的结果，我们认为α 1基因表达的抑制可能与IgAD的发病机制有关。

项目成果

Clinical improvement of diffuse lymphangiomatosis with pegylated interferon alfa-2b therapy: Case report and review of the literature

• DOI: 10.1080/08880010701533603
• 发表时间: 2007-01-01
• 期刊: PEDIATRIC HEMATOLOGY AND ONCOLOGY
• 影响因子: 1.7
• 作者: Ozeki, Michio;Funato, Michinori;Kondo, Naomi
• 通讯作者: Kondo, Naomi

Lymphcyte Responses to Chymotrypsin-or Trypsin V-Digested β-Lactoglobulin in Patients with Cow's Milk Allergy.

牛奶过敏患者淋巴细胞对胰凝乳蛋白酶或胰蛋白酶 V 消化的 β-乳球蛋白的反应。

• 发表时间: 2007
• 期刊: Allergy, Asthma, and Clinical Immunology 3
• 作者: Kondo M;Fukao T;Shinoda S;Kawamoto N;Kaneko H;Kato Z;Matsui E;Teramoto T;Nakano T;Kondo N.
• 通讯作者: Kondo N.

Age-related changes in BAFF and APRIL profiles and up-regulated BAFF and APRIL expression in patients with primary antibody deficiency.

一抗缺陷患者中 BAFF 和 APRIL 谱与年龄相关的变化以及 BAFF 和 APRIL 表达上调。

• 发表时间: 2008
• 期刊: Int J Mol Med 21
• 作者: Jin R;Kaneko H;Suzuki H;Arai T;Teramoto T;Fukao T;Kondo N
• 通讯作者: Kondo N

Identification of Ali-mediated, large deletion-spanning introns 19-26 in PHKA2 in a patient with X-linked liver glycogenosis(hepatic phosphorylase kinase deficiency).

X 连锁肝糖原增多症（肝磷酸化酶激酶缺乏症）患者 PHKA2 中 Ali 介导的大缺失内含子 19-26 的鉴定。

• 发表时间: 2007
• 期刊: Molecular Genetics and Metabolism 92
• 作者: Fukao T;Zhang G;Aoki Y;Arai T;Teramoto T;Kaneko H;Sugie H;Kondo N
• 通讯作者: Kondo N

Age-related changes in BAFF and APRIL profiles and up-regulated BAFF and APRIL expression in patients with primary antibody deficiency

一抗缺陷患者 BAFF 和 APRIL 谱与年龄相关的变化以及 BAFF 和 APRIL 表达上调

• 发表时间: 2008
• 期刊: Int J Mol Med 21
• 作者: Jin;R.;Kaneko;H.;Suzuki;H.;Arai;T.;Teramoto;T.;Fukao;T.;Kondo;N
• 通讯作者: N