抗体

小鼠抗人类C3

MBS225710

0.1毫克

300美元

单克隆

小鼠

未共轭

[AB01-3B4]

抗体

小鼠抗人类C3

[C3]

[CPAMD1]

[Complement component 3; Complement C3; complement C3; complement C3; C3 and PZP-like alpha-2-macroglobulin domain-containing protein 1Cleaved into the following 11 chains:Complement C3 beta chainComplement C3 alpha chainC3a anaphylatoxinAcylation stimulating protein; ASP; Alternative name(s):; C3adesArg]

[C3]

[C3;C3;ASP;C3a;C3b;AHUS5;ARMD9;CPAMD1;HEL-S-62p;CPAMD1;ASP]

CO3_HUMAN

单克隆

IgG1

[AB01-3B4]

小鼠

人类

1663

Purified IgG prepared by affinity chromatography on Protein G

液体;磷酸盐缓冲液

1.0毫克/毫升

Store at 4 degree C or at -20 degree C if preferred. Storage in frost-free freezers is not recommended. This product should be stored undiluted. Avoid repeated freezing and thawing as this may denature the antibody. Should this product contain a precipitate we recommend microcentrifugation before use.

免疫印迹(免疫印迹)

WB: 1:1000. Under reducing conditions the C3 alpha chain at an approximate molecular weight of 115 kDa and the C3c alpha chain fragment 2 at approximately 39 kDa can be detected by Western blotting. Under non reducing conditions the mature C3 band can be detected at a molecular weight of approximately 186 kDa.

试验数据

Immunogen: E Coli-derived recombinant human C3 (Gly1200-Asn1663), Swiss-Prot: P01024

Fusion Partners: Spleen cells from mice immunized with BALB/c were fused with cells of the mouse SP2/0 myeloma cell line. Preservative Stabilizers: 0.09% Sodium Azide (NaN3)

Mouse anti Human C3 antibody, clone AB01-384 recognizes complement C3. C3 is a central component of the classical, alternate and lectin pathways in the complement cascade which forms part of the innate immune defenses. The central position of C3 in the cascade has led to the emergence of immune invasion strategies by many human pathogens, including Staphylococcus aureus which expresses 2 proteins that interfere with the activation of C3 (Garcia et al. 2012). C3 Deficiencies in humans also show an increased susceptibility to bacterial infections in early childhood the development of autoimmune disease and kidney diseases (Ricklin et al. 2016). Conversely, complement over-activation can result in various disease pathologies, including induction of inflammatory pathways causing tissue damage. With complements' role in various diseases, pharmaceutical and biotech companies have targeted the cascade for potential therapeutic targets in a range of immune and inflammatory diseases as well as in transplantation medicine (Ricklin et al. 2012). C3 is activated via its' cleavage into fragments which have varying functions within the complement cascade.

152012784

AAI50180.1

P01024

Activation Of C3 And C5 Pathway (106412); Adaptive Immune System Pathway (366160); Alternative Complement Activation Pathway (106410); Chagas Disease (American Trypanosomiasis) Pathway (147809); Chagas Disease (American Trypanosomiasis) Pathway (147795); Class A/1 (Rhodopsin-like Receptors) Pathway (106357); Complement Activation, Classical Pathway (198823); Complement And Coagulation Cascades Pathway (198880); Complement And Coagulation Cascades Pathway (83073); Complement And Coagulation Cascades Pathway (484)

Complement component C3 plays a central role in the activation of complement system. Its activation is required for both classical and alternative complement activation pathways. The encoded preproprotein is proteolytically processed to generate alpha and beta subunits that form the mature protein, which is then further processed to generate numerous peptide products. The C3a peptide, also known as the C3a anaphylatoxin, modulates inflammation and possesses antimicrobial activity. Mutations in this gene are associated with atypical hemolytic uremic syndrome and age-related macular degeneration in human patients. [provided by RefSeq, Nov 2015]

C3: 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. Defects in C3 are the cause of complement component 3 deficiency (C3D). 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). 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). 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. 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. Increased levels of C3 and its cleavage product ASP, are associated with obesity, diabetes and coronary heart disease. Short-term endurance training reduces baseline ASP levels and subsequently fat storage. Protein type: Secreted, signal peptide; Secreted; Inhibitor. Chromosomal Location of Human Ortholog: 19p13.3-p13.2. Cellular Component: extracellular space; plasma membrane; extracellular region. Molecular Function: protein binding; endopeptidase inhibitor activity; C5L2 anaphylatoxin chemotactic receptor binding; receptor binding. Biological Process: regulation of immune response; complement activation, alternative pathway; signal transduction; fatty acid metabolic process; complement activation; G-protein coupled receptor protein signaling pathway; positive regulation of angiogenesis; positive regulation of activation of membrane attack complex; positive regulation of G-protein coupled receptor protein signaling pathway; positive regulation of type IIa hypersensitivity; regulation of complement activation; innate immune response; immune response; positive regulation of protein amino acid phosphorylation; inflammatory response; complement activation, classical pathway. Disease: Complement Component 3 Deficiency, Autosomal Recessive; Hemolytic Uremic Syndrome, Atypical, Susceptibility To, 5; Macular Degeneration, Age-related, 9

0.1毫克

300美元