原發性免疫缺陷病(PID)—專業代理Bioporto產品

上海惠誠生物物專業代理并分銷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，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。上海惠誠生物提供的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，惠誠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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