原發性免疫缺陷病【PID】研究 -「西寶生物」專業代理Bioporto產品
西寶生物專業代理并分銷BioPorto品牌與原發性免疫缺陷病（PID）補體系統研究相關的單克隆抗體、抗原及ELISA試劑盒。免疫缺陷病是由免疫系統先天性發育障礙或后天損傷而致的一組綜合征。由遺傳因素或先天性免疫系統發育不良造成的免疫功能障礙，稱為原發性免疫缺陷病(PID)。由后天因素(如感染、營養、疾病、藥物等)引起的免疫功能障礙，稱為繼發性免疫缺陷病(SID)或獲得性(acquired)免疫缺陷病。
補體系統是先天性免疫系統的一個組成部分，補體系統由多種不同的血漿蛋白組成，廣泛參與機體微生物防御反應以及免疫調節，也可介導免疫病理的損傷性反應，是體內具有重要生物學作用的效應系統和效應放大系統。
甘露糖結合凝集素(MBL)
甘露糖結合凝集素(mannose binding lectin, MBL)是參加天然免疫的宿主防御分子，它能夠通過凝集素途徑激活補體系統和吞噬作用從而在宿主免疫防御中發揮重要作用。MBL基因多態性與多種疾病易感性，嚴重程度以及預后密切相關，在免疫系統尚未成熟的兒童或同時存在原發或繼發免疫缺陷患者，低表達MBL基因型與感染性疾病，自身免疫病的相關性尤其顯著。西寶生物提供獨特的MBL Oligomer ELISA試劑盒，用于人源血清或血漿中的功能性MBL水平；提供檢測人、雞、馬、大鼠來源的MBL抗體，血清中的人源MBL抗原。
| MBL ELISA 檢測試劑盒 | ||||||
| 貨號 | 產品名稱 | 描述 | 包裝規格 | 檢測種屬 | 檢測樣品 | 檢測范圍 |
| 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 單克隆抗體 | |||||
| 貨號 | 產品名稱 | 規格 | 宿主 | 應用 | |
| 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） | |||||
| 貨號 | 產品名稱 | 包裝規格 | 形式 | 存儲條件 | 保質期 |
| 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 |
其他補體激活途徑相關因子
除MBL以外，西寶生物專業提供其他補體激活途徑相關因子的單克隆抗體：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等抗體。
| 貨號 | 產品名稱 | 規格 | 宿主 | 應用 |
|
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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