catalog number :
MBS212910
products full name :
MOUSE ANTI HUMAN C3c
products short name :
[C3c]
other names :
[complement C3; Complement C3; complement C3; C3 and PZP-like alpha-2-macroglobulin domain-containing protein 1; C3a anaphylatoxin; acylation-stimulating protein cleavage product; complement component C3; complement component C3a; complement component C3b; epididymis secretory sperm binding protein Li 62p; prepro-C3; complement component 3; C3 and PZP-like alpha-2-macroglobulin domain-containing protein 1Cleaved into the following 12 chains:Complement C3 beta chain; C3-beta-c; C3bc; Complement C3 alpha chain; C3a anaphylatoxin; Acylation stimulating protein; ASPAlternative name(s):C3adesArg]
products gene name :
[C3c]
other gene names :
[C3; C3; ASP; C3a; C3b; AHUS5; ARMD9; CPAMD1; HEL-S-62p; CPAMD1; C3bc; ASP]
uniprot entry name :
CO3_HUMAN
form :
Purified. Purified IgG - liquid
concentration :
IgG concentration 1.0mg/ml
storage stability :
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. Shelf Life: 18 months from date of despatch.
tested application :
Immunohistology Frozen, ELISA (EIA)*, Flow cytometry (FC/FACS), Western Blot (WB)
app notes :
ELISA: Application Note: Clone 10-02A binds both C3c and C3 and so is not suitable for all ELISA applications.
other info1 :
Perservative Stabilisers: 0.1% Sodium Azide (NaN3). Preparation: Purified IgG prepared by affinity chromatography on Protein A
other info2 :
Histology Positive Control Tissue: Kidney from post streptococal glomerulonephritis patients. Buffer Solution: Borate buffered saline. Target Species: Human. Immunogen: Human C3 from normal serum, highly purified by chromatography.
products description :
Mouse anti human C3c, clone 10-02A, recognizes 135kDa complement component 3c present in blood serum. 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.
ncbi acc num :
NP_000055.2
ncbi gb acc num :
NM_000064.3
ncbi mol weight :
187,148 Da
ncbi pathways :
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)
ncbi summary :
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. A peptide (C3a) derived from the encoded protein has antimicrobial activity, so people with C3 deficiency are susceptible to bacterial infection. [provided by RefSeq, Nov 2014]
uniprot summary :
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: Inhibitor; Secreted; Secreted, signal peptide. 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