FITC标记的补体C1qγ链多肽抗体
产品名称： FITC标记的补体C1qγ链多肽抗体
英文名称： Anti-C1QC/FITC
产品编号： HZ-11337R-FITC
产品价格： null
产品产地： 中国/上海
品牌商标： HZbscience
更新时间： 2023-08-17T10:24:20
使用范围： ICC=1:50-200 IF=1:50-200
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Rabbit Anti-C1QC/FITC Conjugated antibody
FITC标记的补体C1qγ链多肽抗体
| 产品编号 | bs-11337R-FITC |
| 英文名称 | Anti-C1QC/FITC |
| 中文名称 | FITC标记的补体C1qγ链多肽抗体 |
| 别 名 | C1Q C; C1qc; C1QC_HUMAN; C1QG; Complement C1q subcomponent subunit C; Complement component 1, q subcomponent, C chain; complement component 1, q subcomponent, gamma polypeptide; AI385742. |
| 规格价格 | 100ul/2980元 购买 大包装/询价 |
| 说 明 书 | 100ul |
| 研究领域 | 免疫学 神经生物学 细胞类型标志物 |
| 抗体来源 | Rabbit |
| 克隆类型 | Polyclonal |
| 交叉反应 | Human, Mouse, Rat, Dog, Horse, Rabbit, |
| 产品应用 | ICC=1:50-200 IF=1:50-200 not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 分 子 量 | 23kDa |
| 性 状 | Lyophilized or Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human C1QC |
| 亚 型 | IgG |
| 纯化方法 | affinity purified by Protein A |
| 储 存 液 | 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. |
| 保存条件 | Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C. |
| 产品介绍 | background: C1q, a subcomponent of the classical complement pathway, is composed of nine subunits that mediate classical complement activation and thereby play an important role in the immune response. Six of these subunits are disulfide-linked dimers of chains A and B, while three of these subunits, designated C1q-A through C1q-C, are disulfide-linked dimers of chain C. The presence of receptors for C1q on effector cells modulates its activity, which may be antibody-dependent or independent. Macrophages are the primary source of C1q, while anti-inflammatory drugs as well as cytokines differentially regulate expression of the mRNA, as well as the protein. However, its ability to modulate the interaction of platelets with collagen and immune complexes suggests C1q influences homeostasis as well as other immune activities, and perhaps thrombotic complications resulting from immune injury. Defects in C1q-A, C1q-B and C1q-C cause inactivation of the classical pathway, leading to a rare genetic disorder characterized by lupus-like symptoms. Function: C1q associates with the proenzymes C1r and C1s to yield C1, the first component of the serum complement system. The collagen-like regions of C1q interact with the Ca(2+)-dependent C1r(2)C1s(2) proenzyme complex, and efficient activation of C1 takes place on interaction of the globular heads of C1q with the Fc regions of IgG or IgM antibody present in immune complexes. Subunit: C1 is a calcium-dependent trimolecular complex of C1q, R and S in the molar ration of 1:2:2. C1q subcomponent is composed of nine subunits, six of which are disulfide-linked dimers of the A and B chains, and three of which are disulfide-linked dimers of the C chain. Subcellular Location: Secreted. Post-translational modifications: O-linked glycans consist of Glc-Gal disaccharides bound to the oxygen atom of post-translationally added hydroxyl groups. DISEASE: Defects in C1QC are a cause of complement component C1q deficiency (C1QD) [MIM:613652]. A rare defect resulting in C1 deficiency and impaired activation of the complement classical pathway. C1 deficiency generally leads to severe immune complex disease with features of systemic lupus erythematosus and glomerulonephritis. Similarity: Contains 1 C1q domain. Contains 1 collagen-like domain. Database links: Entrez Gene: 714 Human Entrez Gene: 12262 Mouse Entrez Gene: 362634 Rat Omim: 120575 Human SwissProt: P02747 Human SwissProt: Q02105 Mouse SwissProt: P31722 Rat Unigene: 467753 Human Unigene: 439732 Mouse Unigene: 2393 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications |
C1q是经典补体途径的一个亚组分，由介导经典补体激活的九个亚基组成，从而在免疫应答中发挥重要作用。这些亚基中的六个是链A和B的二硫键连接的二聚体，而这些亚基中的三个通过C1q-C被命名为C1Q-A，是链C的二硫键连二聚体。效应细胞上C1q的受体的存在调节其活性，其可以是抗体依赖的或独立的。巨噬细胞是C1q的主要来源，而抗炎药物以及细胞因子差异调节mRNA和蛋白质的表达。然而，其调节血小板与胶原和免疫复合物的相互作用的能力提示C1q影响体内平衡以及其他免疫活动，可能是由免疫损伤引起的血栓性并发症。C1Q-A、C1Q-B和C1Q-C中的缺陷导致经典途径的失活，导致以狼疮样症状为特征的罕见遗传障碍。