原发性免疫缺陷病【PID】研究 -「西宝生物」专业代理Bioporto产品
西宝生物专业代理并分销BioPorto品牌与原发性免疫缺陷病（PID）补体系统研究相关的单克隆抗体、抗原及ELISA试剂盒。免疫缺陷病是由免疫系统先天性发育障碍或后天损伤而致的一组综合征。由遗传因素或先天性免疫系统发育不良造成的免疫功能障碍，称为原发性免疫缺陷病(PID)。由后天因素(如感染、营养、疾病、药物等)引起的免疫功能障碍，称为继发性免疫缺陷病(SID)或获得性(acquired)免疫缺陷病。
补体系统是先天性免疫系统的一个组成部分，补体系统由多种不同的血浆蛋白组成，广泛参与机体微生物防御反应以及免疫调节，也可介导免疫病理的损伤性反应，是体内具有重要生物学作用的效应系统和效应放大系统。
甘露糖结合凝集素(MBL)
甘露糖结合凝集素(mannose binding lectin, MBL)是参加天然免疫的宿主防御分子，它能够通过凝集素途径激活补体系统和吞噬作用从而在宿主免疫防御中发挥重要作用。MBL基因多态性与多种疾病易感性，严重程度以及预后密切相关，在免疫系统尚未成熟的儿童或同时存在原发或继发免疫缺陷患者，低表达MBL基因型与感染性疾病，自身免疫病的相关性尤其显著。西宝生物提供独特的MBL Oligomer ELISA试剂盒，用于人源血清或血浆中的功能性MBL水平；提供检测人、鸡、马、大鼠来源的MBL抗体，血清中的人源MBL抗原。
| MBL ELISA 检测试剂盒 | ||||||
| 货号 | 产品名称 | 描述 | 包装规格 | 检测种属 | 检测样品 | 检测范围 |
| KIT 029 | MBL Oligomer ELISA Kit | An ELISA for the determination of oligomerized MBL in human serum or heparin plasma | 96 well | Human | Urine; plasma; serum; tissue extracts or culture media | 0.50-40 ng/mL |
| MBL 单克隆抗体 | |||||
| 货号 | 产品名称 | 规格 | 宿主 | 应用 | |
| HYB 131-01-02 | Mannan-binding lectin (human, MBL) | 200µl | Mouse | ELISA, WB, IHC-P, IHC-F | |
| HYB 131-01-1 | Mannan-binding lectin (human, MBL) | 1ml | Mouse | ELISA, WB, IHC-P, IHC-F | |
| HYB 131-01B-005 | Mannan-binding lectin (human, MBL) | 50µl | Mouse | ELISA, WB | |
| HYB 131-10-02 | Mannan-binding lectin (human, MBL) | 200µl | Mouse | ELISA, WB | |
| HYB 131-10-1 | Mannan-binding lectin (human, MBL) | 1ml | Mouse | ELISA, WB | |
| HYB 131-11-02 | Mannan-binding lectin (human, MBL) | 200µl | Mouse | ELISA, WB, IP | |
| HYB 131-11-1 | Mannan-binding lectin (human, MBL) | 1ml | Mouse | ELISA, WB, IP | |
| HYB 131-14-02 | Mannan-binding lectin (human, horse, pig, MBL) | 200µl | Mouse | ELISA, WB | |
| HYB 131-14-1 | Mannan-binding lectin (human, horse, pig, MBL) | 1ml | Mouse | ELISA, WB | |
| HYB 131-18-02 | Mannan-binding lectin (rat, MBL) | 200µl | Mouse | ELISA, WB | |
| HYB 131-18-1 | Mannan-binding lectin (rat, MBL) | 1ml | Mouse | ELISA, WB | |
| HYB 182-01-02 | Mannan-binding lectin (chicken, MBL) | 200µl | Mouse | ELISA, WB, IHC | |
| HYB 182-01-1 | Mannan-binding lectin (chicken, MBL) | 1ml | Mouse | ELISA, WB, IHC | |
| MBL 抗原（血清） | |||||
| 货号 | 产品名称 | 包装规格 | 形式 | 存储条件 | 保质期 |
| SER 101 | MBL standard serum (human) - 1000 (AU) | 1mL | Freeze-dried, undiluted pooled human serum | -20°C | 2 years |
| SER 103 | MBL oligomer deficient serum, B/C genotype (human) | 1mL | Frozen, undiluted human serum | -20°C | 2 years |
其他补体激活途径相关因子
除MBL以外，西宝生物专业提供其他补体激活途径相关因子的单克隆抗体：Complement C1-INH、C1s、C2、C3、C3d、C3a/C3a（desArg）、C9、C5、C5a/C5a（desArg）、C5b-9、C4；L-ficolin、H-ficolin、M-Ficolin；Factor D、Factor H、Factor B，Properdin，Glycoprotein-340等抗体。
| 货号 | 产品名称 | 规格 | 宿主 | 应用 |
|
ABS 002-09-02 |
Complement component C1s (human, pro- and activated enzyme) |
200µl |
Mouse |
ELISA |
| ABS 002-09-1 | Complement component C1s (human, pro- and activated enzyme) | 1ml | Mouse | ELISA |
| ABS 002-49-02 | Complement component C1s (human, pro- and activated enzyme) | 200µl | Mouse | ELISA |
| ABS 002-49-1 | Complement component C1s (human, pro- and activated enzyme) | 1ml | Mouse | ELISA |
| ABS 004-22-02 | Complement component C9 (human) | 200µl | Mouse | ELISA, WB |
| ABS 004-22-1 | Complement component C9 (human) | 1ml | Mouse | ELISA, WB |
| ABS 004-53-02 | Complement component C9 (human) | 200µl | Mouse | ELISA |
| ABS 004-53-1 | Complement component C9 (human) | 1ml | Mouse | ELISA |
| CAM 008-01-02 | Complement Component C3 (human) | 200µl | Mouse | ELISA, IP, IAP |
| CAM 008-01-1 | Complement Component C3 (human) | 1ml | Mouse | ELISA, IP, IAP |
| CAM 072-18-02 | Complement C4c | 200µl | Mouse | ELISA, WB, IP |
| CAM 072-18-1 | Complement C4c | 1ml | Mouse | ELISA, WB, IP |
| DIA 011-01-02 | Complement component C5b-9 | 200µl | Mouse | ELISA, WB (not applicable), IHC-F, IHC-P |
| DIA 011-01-1 | Complement component C5b-9 | 1ml | Mouse | ELISA, WB (not applicable), IHC-F, IHC-P |
| DIA 011-01B-01 | Complement component C5b-9 | 100µl | Mouse | ELISA |
| GAU 008-01-02 | Complement factor D (human) | 200µl | Mouse | ELISA, WB |
| GAU 008-01-1 | Complement factor D (human) | 1ml | Mouse | ELISA, WB |
| GAU 008-01B-01 | Complement factor D (human) | 100µl | Mouse | ELISA |
| GAU 010-04-02 | Complement factor D (human) | 200µl | Mouse | ELISA, WB |
| GAU 010-04-1 | Complement factor D (human) | 1ml | Mouse | ELISA, WB |
| GAU 013-16-02 | Complement component C3a/C3a(desArg)/C3 (human) | 200µl | Mouse | ELISA, WB |
| GAU 013-16-1 | Complement component C3a/C3a(desArg)/C3 (human) | 1ml | Mouse | ELISA, WB |
| GAU 017-01-02 | Complement component C3a/C3a(desArg)/C3 (human) | 200µl | Mouse | ELISA, WB |
| GAU 017-01-1 | Complement component C3a/C3a(desArg)/C3 (human) | 1ml | Mouse | ELISA, WB |
| GAU 017-01B-01 | Complement component C3a/C3a(desArg)/C3 (human) | 100µl | Mouse | ELISA |
| GAU 018-03-02 | Complement factor H (human, ß1H-globulin) | 200µl | Mouse | ELISA, WB, IHC |
| GAU 018-03-1 | Complement factor H (human, ß1H-globulin) | 1ml | Mouse | ELISA, WB, IHC |
| GAU 020-03-02 | Complement factor H (human, ß1H-globulin) | 200µl | Mouse | ELISA, WB |
| GAU 020-03-1 | Complement factor H (human, ß1H-globulin) | 1ml | Mouse | ELISA, WB |
| GAU 020-03B-01 | Complement factor H (human, ß1H-globulin) | 100µl | Mouse | ELISA |
| GAU 025-05-02 | Anti-Complement component C5a/C5a(desArg) anaphylatoxin (human) | 200µl | Mouse | ELISA, WB |
| GAU 025-05-1 | Anti-Complement component C5a/C5a(desArg) anaphylatoxin (human) | 1ml | Mouse | ELISA, WB |
| HAV 003-05-02 | Complement component C3 (human) | 200µl | Mouse | ELISA, WB |
| HAV 003-05-1 | Complement component C3 (human) | 1ml | Mouse | ELISA, WB |
| HAV 004-01-02 | Complement component C3 (human), beta-chain genetic variant | 200µl | Mouse | ELISA, WB |
| HAV 004-01-1 | Complement component C3 (human), beta-chain genetic variant | 1ml | Mouse | ELISA, WB |
| HYB 005-01-02 | Complement component C3 (human) | 200µl | Mouse | ELISA, WB |
| HYB 005-01-1 | Complement component C3 (human) | 1ml | Mouse | ELISA, WB |
| HYB 008-02-02 | Complement factor B (human) | 200µl | Mouse | ELISA, WB |
| HYB 008-02-1 | Complement factor B (human) | 1ml | Mouse | ELISA, WB |
| HYB 008-06-02 | Complement factor B (human) | 200µl | Mouse | ELISA, WB |
| HYB 008-06-1 | Complement factor B (human) | 1ml | Mouse | ELISA, WB |
| HYB 008-06B-01 | Complement factor B (human) | 100µl | Mouse | ELISA |
| HYB 029-02-02 | Complement component C5 (human) | 200µl | Mouse | ELISA, WB |
| HYB 029-02-1 | Complement component C5 (human) | 1ml | Mouse | ELISA, WB |
| HYB 029-03-02 | Complement component C5 (human) | 200µl | Mouse | ELISA, WB |
| HYB 029-03-1 | Complement component C5 (human) | 1ml | Mouse | ELISA, WB |
| HYB 030-08-02 | Complement component C3d (human) | 200µl | Mouse | ELISA, WB |
| HYB 030-08-1 | Complement component C3d (human) | 1ml | Mouse | ELISA, WB |
| HYB 050-04-02 | Complement component C2 (human) | 200µl | Mouse | ELISA, WB |
| HYB 050-04-1 | Complement component C2 (human) | 1ml | Mouse | ELISA, WB |
| HYB 050-05-02 | Complement component C2 (human) | 200µl | Mouse | ELISA, WB |
| HYB 050-05-1 | Complement component C2 (human) | 1ml | Mouse | ELISA, WB |
| HYB 050-08-02 | Complement component C2 (human) | 200µl | Mouse | ELISA, WB |
| HYB 050-08-1 | Complement component C2 (human) | 1ml | Mouse | ELISA, WB |
| HYB 061-01-02 | Complement factor I (human) | 200µl | Mouse | ELISA, WB |
| HYB 061-01-1 | Complement factor I (human) | 1ml | Mouse | ELISA, WB |
| HYB 118-02-02 | Complement component C3 (rat) | 200µl | Mouse | ELISA, IHC |
| HYB 118-02-1 | Complement component C3 (rat) | 1ml | Mouse | ELISA, IHC |
| HYB 162-02-02 | Complement component C4 (human) | 200µl | Mouse | ELISA, WB |
| HYB 162-02-1 | Complement component C4 (human) | 1ml | Mouse | ELISA, WB |
| HYB 162-02B-01 | Anti-Complement Component C4 | 100µl | Mouse | ELISA |
| HYB 162-04-02 | Complement component C4 (human) | 200µl | Mouse | ELISA, WB |
| HYB 162-04-1 | Complement component C4 (human) | 1ml | Mouse | ELISA, WB |
| HYB 268-01-02 | Complement factor H (human, ß1H-globulin) | 200µl | Mouse | ELISA, WB |
| HYB 268-01-1 | Complement factor H (human, ß1H-globulin) | 1ml | Mouse | ELISA, WB |
| HYB 268-01B-01 | Complement factor H (human, ß1H-globulin) | 100µl | Mouse | ELISA |
| HYB 288-05-02 | Complement component C1-inhibitor (C1-INH) | 200µl | Mouse | ELISA |
| HYB 288-05-1 | Complement component C1-inhibitor (C1-INH) | 1ml | Mouse | ELISA |
| ABS 005-16-02 | L-Ficolin (human) | 200µl | Mouse | ELISA, WB |
| ABS 005-16-1 | L-Ficolin (human) | 1ml | Mouse | ELISA, WB |
| ABS 005-19-02 | L-Ficolin (human) | 200µl | Mouse | ELISA, WB, IP |
| ABS 005-19-1 | L-Ficolin (human) | 1ml | Mouse | ELISA, WB, IP |
| ABS 036-01-02 | M-Ficolin (human) | 200µl | Mouse | ELISA, IHC |
| ABS 036-01-1 | M-Ficolin (human) | 1ml | Mouse | ELISA, IHC |
| ABS 036-05-02 | M-Ficolin (human) | 200µl | Mouse | ELISA |
| ABS 036-05-1 | M-Ficolin (human) | 1ml | Mouse | ELISA |
| RIG 334-01-02 | Ficolin H (human) | 200µl | Mouse | ELISA, IP |
| RIG 334-01-1 | Ficolin H (human) | 1ml | Mouse | ELISA, IP |
| RIG 334-01B-01 | Ficolin H (human) | 100µl | Mouse | ELISA |
| HYB 039-04-02 | Properdin (human) | 200µl | Mouse | ELISA, WB |
| HYB 039-04-1 | Properdin (human) | 1ml | Mouse | ELISA, WB |
| HYB 039-04B-01 | Properdin (human) | 100µl | Mouse | ELISA |
| HYB 213-01-02 | Glycoprotein-340 (human, gp-340) | 200µl | Mouse | ELISA, WB, IHC |
| HYB 213-01-1 | Glycoprotein-340 (human, gp-340) | 1ml | Mouse | ELISA, WB, IHC |
| HYB 213-06-02 | Glycoprotein-340 (human, gp-340) | 200µl | Mouse | ELISA, WB, IHC |
| HYB 213-06-1 | Glycoprotein-340 (human, gp-340) | 1ml | Mouse | ELISA, WB, IHC |
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