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背景資料

C1q is the first component of the classical pathway of complement activation. C1q along with the enzymatically active components C1r and C1s forms the C1 complex. When C1 binds to immunoglobulins in the form of immune complexes， it leads to activation of C1r and C1s proteases and a further activates the classical pathway of complement.
C1q is a glycoprotein that belongs to the collectin family， having a molecular weight of about 410-462 kDa. C1q is a hexamer composed of globular heads attached to collagen-like triple-helix tails. The globular heads of C1q exclusively bind to the CH2 domain of IgG molecules or the CH3 domain of IgM. Each heavy chain of the immunoglobulin molecule contains a single binding site for C1q. Given that C1q must bind to no less than two heavy chains in order to alter its conformation and activate C1r and C1s， its activation follows only after binding to immunoglobulins in the form of immune complexes bound to m*lent antigens. C1q’s main physiological role is in the clearance of immune complexes and apoptotic bodies from the organism. Interruption of this process may lead to development of autoimmunity. Individuals with genetic deficiencies of C1q or other components of the classical pathway are at risk to develop SLE. C1q specifically binds to apoptotic bodies of human keratinocytes， vascular endothelial cells and lymphocytes. Complement components C1q and bound C3 mediate the clearance of apoptotic bodies. Hence， C1q may advance the clearance of autoantigens， avoiding stimulation of the immune system. Nonetheless， an extended exposition of the immune system to the neoepitope exposed on C1q molecules bound to immune complexes or apoptotic bodies could ultimately lead to an autoimmune response against C1q itself and to an altered complement function. C1q deficiency may also lead to disruption of the negative selection of autoreactive B cells. C1q along with other specific recognition proteins bind to the highly conserved lupus antigens （dsDNA and nuclear proteins） and activate the complement system. Autoantibodies against C1q （anti-C1q） are found in a number of autoimmune and infectious diseases like glomerulonephritis （GN） and lupus erythematosus （SLE）， these antibodies are significant in clinical practice due to their negative predictive value.

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來源宿主

人血漿中提取純化

溶解建議

Sterile Filtered White lyophilized （freeze-dried） powder. Lyophilized from 0.01M Na2 EDTA solution containing 0.3M NaCl pH 7.5. It is recommended to reconstitute the lyophilized C1q in sterile 18MΩ-cm H2O.

保存建議

Human C1q although stable at room temperature for 3 weeks， should be stored between 2-8℃.

其他

ProSpec 是一家全球著名細胞因子蛋白生產和研發的公司，具有20多年的生產經驗，專注于蛋白（重組及合成）生產研發。我們的核心產品包括：干擾素、白介素、骨蛋白、腫瘤壞死因子、成纖維生長因子、瘦素、干細胞因子、催乳素、表皮生長因子、胰島素樣生長因子、神經生長因子、集落刺激因子、激素、酶、病毒抗原以及各種重組蛋白等。  生產工藝和規模使其可以提供毫克到克級蛋白，價格優于同類公司。絕大部分產品是天然成熟型蛋白，而不含有標簽蛋白。考慮到大部分研究者希望能更靈活地配制蛋白溶液，ProSpec-Tany絕大部分產品沒有添加保護劑或鹽，這就對工藝提出了更高的要求。同時，ProSpec-Tany絕大部分產品為凍干粉，因此易于運輸和保存。