Pathogenesis and clinical course of primary antibody deficiency

一抗缺陷的发病机制和临床过程

• 批准号: 20591250
• 负责人: KANEKO Hideo
• 金额: $ 3万
• 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: 2008
• 资助国家: 日本
• 起止时间: 2008 至 2010
• 项目状态: 已结题
• 来源: https://kaken.nii.ac.jp/grant/KAKENHI-PROJECT-20591250/
• 关键词: 高IgM症候群; activation induced cytidine deaminase; R190X変異; Dominant negative mutation; 体細胞突然変異; AID; 常染色体優性遺伝; 記憶B細胞; XLA; 自然免疫; ヘリコバクター感染症; 胸膜炎; 高エンドトキシン血症; IgA欠損症; IgAサブクラス; IgA1定常部欠失; partial IgA deficiency

The pattern of serum Ig level were different between mother and daughter with same AID mutation (R190X). The immune phenotype of HIGM2 (R190X) might be affected by the environmental factors or the other genetic factors. In HIGM2 (R190X), specific antibody of IgM class was expressed for long time by IgM memory B cells, in which have done somatic hypermutation. In HIGM2 (R190X), some B cell could switched IgM to IgG. This switched B cell might not survive for long time and IgG antibody might become disappeared.

相同AID突变(R190X)的母女血清Ig水平模式不同。HIGM2(R190X)的免疫表型可能受环境因素或其他遗传因素的影响。在HIGM2(R190X)中，发生过体细胞高突变的IgM记忆B细胞长期表达特异性的IgM抗体。在HIGM2(R190X)中，部分B细胞可将IgM转换为IgG。这种转换的B细胞可能不会存活很长时间，而且抗体可能会消失。

项目成果

The response of bovine beta-lactoglobulin-specific T-cell clones to single amino acid substituti on of T-cell core epitope

牛β-乳球蛋白特异性T细胞克隆对T细胞核心表位单氨基酸取代的反应

• 期刊: Pediatrric Allergy and Immunology (In press)
• 作者: Kondo M;Kaneko H;Fukao T;Suzuki K;Sakaguchi H;Shinoda S;Kato Z;Matsui E;Teramoto T;Nakano T;Kondo N
• 通讯作者: Kondo N

Age-related changes of transforming growth factor betal in Japanese children.

日本儿童转化生长因子β的年龄相关变化。

• 发表时间: 2009
• 期刊: Allergol Int. 58
• 作者: Morimoto M;Matsui E;Kawamoto N;Sakurai S;Kaneko H;Fukao T;Iwasa S;Shiraki M;Kasahara K;Kondo N.
• 通讯作者: Kondo N.

A Novel Polymorphism in the 5-Iipoxygenase Gene Associated with Bronchial Asthma in Japanese Children.

与日本儿童支气管哮喘相关的 5-脂加氧酶基因的新多态性。

• 发表时间: 2008
• 期刊: Int J Mol Med 21
• 作者: Bai CY;Matsui E;Ohnishi H;Kimata K;Kasahara K;Kaneko H;Katoz;Fukao T;Kondo N
• 通讯作者: Kondo N

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

• DOI: 10.3892/ijmm.21.2.233
• 发表时间: 2008-02
• 期刊: International journal of molecular medicine
• 影响因子: 5.4
• 作者: R. Jin;H. Kaneko;Hiroko Suzuki;T. Arai;Takahide Teramoto;T. Fukao;N. Kondo

Oral Administration of the Thyrotropin-Releasing Hormone (TRH) Analogue, Taltireline Hydrate, in Spinal Muscular Atrophy

• DOI: 10.1177/0883073809333535
• 发表时间: 2009-08
• 期刊: Journal of Child Neurology
• 影响因子: 1.9
• 作者: Zenichiro Kato;M. Okuda;Y. Okumura;T. Arai;Takahide Teramoto;M. Nishimura;H. Kaneko;N. Kondo