Product Description

Native human C6 is a naturally glycosylated (11%) protein composed of a single polypeptide chain of 105,000 Da.C6 is essential for formation of the membrane attack complex (MAC) and is activated non-proteolytically by binding to recently-formed C5b at the cell membrane.Each pathway of complement activation generates proteolytic enzyme complexes (C3/C5 convertases) which are bound to the target surface (Law, S.K.A. and Reid, K.B.M. (1995); Ross, G.D. (1986)).These enzymes cleave a peptide bond in the larger alpha chain of C5 releasing the anaphylatoxin C5a and activating C5b.This is the only proteolytic step in the assembly of the C5b-9 complex.C5b is unstable, but it remains bound to the activating complex for a brief time (~2 min) during which it either binds a single C6 from the surrounding fluid or it decays and is no longer capable of forming MAC.The C5b,6 complex may also remain bound to the C3/C5 convertase where the binding of a single C7 exposes a membrane-binding region and C5b,6,7 can partially insert into the bilipid layer of the target cell.Up to this point the complex may diffuse away from the target cell and enter the membrane of a nearby cell.This is called bystander lysis or “reactive lysis” and can be a significant source of pathology.Each C5b-7 complex can bind one C8 protein molecule which results in the complex inserting more firmly into the membrane.This complex binds C9 and each bound C9 can bind another C9 initiating formation of a ring structure containing up to 18 C9 molecules (Podack, E.R. (1984)).C5b-9 complexes with one or more C9 are referred to as the Membrane Attack Complex (MAC) of complement.Not all C5b-8 complexes have complete rings of C9 with the average being only three C9 per C5b-8 complex.Completed protein rings of C9 form the pores seen on electron micrographs and they result in leakage of metabolites and small proteins out of the cell as well as movement of water into the cell.If sufficient numbers are inserted into a cell membrane then water flowing into the cell, due to osmotic pressure, will rupture the cell membrane allowing the entire contents of the target cell (or a bystander cell) to be released.Either process may result in cell death.Originally it was thought that this required only one C5b-9 complex per cell (referred to as the “one hit theory” of lysis (Rommel F.A. and Mayer, M.M. (1973)), but this is probably not correct.For example, an erythrocyte requires ~850 C5b-9 complexes, as measured by the number of C7 molecules, for lysis to occur (Bauer, J. et al. (1979)).Host cells protected from MAC by CD59 require sufficient numbers of C5b-9 to tie up all the CD59 and then ~850 C5b-9 in addition.Lysis of nucleated cells requires many more C5b-9 complexes due to their size and due to the presence of multiple defense mechanisms in such cells.

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Complement Technology的C1是级联反应中的第一个补体成分，称为补体经典途径。C1与抗原结合的抗体（免疫复合物）结合并被其激活，从而产生引发级联反应的蛋白酶。C1实际上是钙依赖性复合物中结合在一起的三种不同蛋白质（C1q，C1r和C1s）的非共价复合物。C1q通过其六个臂中的两个或多个与IgG或IgM的Fc结构域结合。多臂与免疫复合物的结合被认为会引入压力，从而导致复合物中的两个C1r蛋白（蛋白酶酶原）自身激活，从而产生两种活性的C1r丝氨酸蛋白酶（Morikis，D.和Lambris，JD（2005））。 。这些激活的C1r亚基裂解并激活复合物中的两个C1s蛋白酶酶原。活化的C1裂解补体成分C4，释放出C4a，并启动C4b与活化表面的共价连接。活化的C1也切割C2，并且C2的较大片段结合至表面附着的C4b，形成C4b，C2a，其为经典途径的C3 / C5转化酶。