The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
生物活性ab96038 contains the intact Beta chain and the Alpha chain without the C3dg, C3a and C3f peptides. This material is suitable for raising polyclonal antiserum to C3.
% . ab96038 is >99% pure by SDS Page.
We do not carry out mass spectrometry analysis to confirm this since we sell our C3 on the basis of their immunological activity.
补充说明Starting material donor tested and found negative for HIV I and II antibodies, Hepatitis B surface antigen, and Hepatitis C antibodies.ab96038 contains the intact Beta chain and the Alpha chain without the C3dg, C3a and C3f peptides. This material is suitable for raising polyclonal antiserum to C3.
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Shipped on dry ice. Upon delivery aliquot and store at -80ºC. Avoid freeze / thaw cycles.
C3 and PZP-like alpha-2-macroglobulin domain-containing protein 1
Complement C3c alpha' chain fragment 2
Complement component 3
Complement component C3
Complement component C3a
Complement component C3b
Complement factor 3
功能C3 plays a central role in the activation of the complement system. Its processing by C3 convertase is the central reaction in both classical and alternative complement pathways. After activation C3b can bind covalently, via its reactive thioester, to cell surface carbohydrates or immune aggregates. Derived from proteolytic degradation of complement C3, C3a anaphylatoxin is a mediator of local inflammatory process. It induces the contraction of smooth muscle, increases vascular permeability and causes histamine release from mast cells and basophilic leukocytes.
疾病相关Defects in C3 are the cause of complement component 3 deficiency (C3D) [MIM:613779]. A rare defect of the complement classical pathway. Patients develop recurrent, severe, pyogenic infections because of ineffective opsonization of pathogens. Some patients may also develop autoimmune disorders, such as arthralgia and vasculitic rashes, lupus-like syndrome and membranoproliferative glomerulonephritis. Genetic variation in C3 is associated with susceptibility to age-related macular degeneration type 9 (ARMD9) [MIM:611378]. ARMD is a multifactorial eye disease and the most common cause of irreversible vision loss in the developed world. In most patients, the disease is manifest as ophthalmoscopically visible yellowish accumulations of protein and lipid that lie beneath the retinal pigment epithelium and within an elastin-containing structure known as Bruch membrane. Defects in C3 are a cause of susceptibility to hemolytic uremic syndrome atypical type 5 (AHUS5) [MIM:612925]. An atypical form of hemolytic uremic syndrome. It is a complex genetic disease characterized by microangiopathic hemolytic anemia, thrombocytopenia, renal failure and absence of episodes of enterocolitis and diarrhea. In contrast to typical hemolytic uremic syndrome, atypical forms have a poorer prognosis, with higher death rates and frequent progression to end-stage renal disease. Note=Susceptibility to the development of atypical hemolytic uremic syndrome can be conferred by mutations in various components of or regulatory factors in the complement cascade system. Other genes may play a role in modifying the phenotype.
翻译后修饰C3b is rapidly split in two positions by factor I and a cofactor to form iC3b (inactivated C3b) and C3f which is released. Then iC3b is slowly cleaved (possibly by factor I) to form C3c (beta chain + alpha' chain fragment 1 + alpha' chain fragment 2), C3dg and C3f. Other proteases produce other fragments such as C3d or C3g. Phosphorylation sites are present in the extracelllular medium.