antibody

Complement C3, C3c (Complement Component C3c, C3/C3c, Acylation Stimulating Protein Cleavage Product, ASP, Complement Component 3, Complement Factor 3, CPAMD1)

MBS632462

0.1 mg

610 USD

monoclonal

mouse

nonconjugated

10B386

western blot, ELISA, immunohistochemistry, flow cytometry, FACS, enzyme immunoassay

Antibody

Complement C3, C3c (Complement Component C3c, C3/C3c, Acylation Stimulating Protein Cleavage Product, ASP, Complement Component 3, Complement Factor 3, CPAMD1)

Complement C3, C3c

Anti -Complement C3, C3c (Complement Component C3c, C3/C3c, Acylation Stimulating Protein Cleavage Product, ASP, Complement Component 3, Complement Factor 3, CPAMD1)

complement C3; Complement C3; complement C3; OTTHUMP00000197086; complement component C3; acylation-stimulating protein cleavage product; C3 and PZP-like alpha-2-macroglobulin domain-containing protein 1; complement component 3; C3 and PZP-like alpha-2-macroglobulin domain-containing protein 1

C3; C3; ASP; AHUS5; ARMD9; CPAMD1; CPAMD1

CO3_HUMAN

Monoclonal

IgG1

10B386

Mouse

Human

Recognizes human complement component 3c (C3c) at ~135kD.

Affinity Purified. Purified by Protein A affinity chromatography.

Supplied as a liquid in borate buffered saline, pH 8.4, 0.09% sodium azide.

1mg/ml

May be stored at 4 degree C for short-term only. For long-term storage and to avoid repeated freezing and thawing, aliquot and store at -20 degree C. Aliquots are stable for at least 12 months at -20 degree C. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Further dilutions can be made in assay buffer.

ELISA (EL/EIA), Western Blot (WB), Immunohistochemistry (IHC), Flow Cytometry (FC/FACS)

ELISA: Binds both C3c and C3 so it is not suitable for all ELISA applications. Immunohistochemistry: Frozen sections. Optimal dilutions to be determined by the researcher. Positive Control for Immunohistochemistry: Kidney from post streptococal glomerulonephritis patients.

Immunogen: Human C3 from normal serum.

Antibodies; Abs to Complement

C3c is generated over the course of complement activation, where convertase C4b2a (classical pathway) and convertase C3bBb (alternative pathway) cleave C3 to C3b and C3a. C3b is further degraded into iC3b and C3dg/C3d. iC3b is then slowly cleaved (possibly by factor I) to form C3c (beta chain + alpha' chain fragment 1 + alpha' chain fragment 2), C3dg and C3f. C3 is crucial in the induction of tolerance generated when an antigen is introduced into immunoprivileged sites and this is exploited by pathogens and cancer cells to evade the immune system by inhibiting complement activation.

115298678

NP_000055.2

NM_000064.2

P01024

187,148 Da

Activation Of C3 And C5 Pathway (106412); Adaptive Immunity Signaling 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. People with C3 deficiency are susceptible to bacterial infection. [provided by RefSeq]

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; Secreted, signal peptide; Inhibitor. Chromosomal Location of Human Ortholog: 19p13.3-p13.2. Cellular Component: extracellular space; extracellular region; plasma membrane. 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 type IIa hypersensitivity; positive regulation of G-protein coupled receptor protein signaling pathway; 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 mg

610 USD