protein

NATIVE HUMAN C3b

MBS238029

0.1 mg

675 USD

Antigen

NATIVE HUMAN C3b

C3b

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

C3b

C3; C3; ASP; C3a; C3b; AHUS5; ARMD9; CPAMD1; HEL-S-62p; CPAMD1; C3bc; ASP

CO3_HUMAN

1663

>90% by SDS-PAGE

Purified. Purified C3b from human serum - liquid

Approximate Protein Concentration: 1.0 mg/ml

Store at -20 degree C only. Storage in frost-free freezers is not recommended. This product should be stored undiluted. Avoid repeated freezing and thawing as this may denature the protein. Should this product contain a precipitate we recommend microcentrifugation before use. Shelf Life: 12 months from date of despatch.

ELISA (EIA)

Preservative Stablizer: None present.

Buffer Solution: Phosphate buffered saline. Target Species: Human. Health and Safety Information: Donor material tested and found negative for HBsAg, HIV antibodies and HCV antibodies. As no test can completely guarantee this material to be free of pathogens it should be handled as potentially infecious.

Cleavage of the C3-alpha chain at peptide bond 77 results in production of C3a and C3b fragments. The C3b fragment is a glycoprotein composed of the modified C3-alpha chain and the intact C3-beta chain. Nascent C3b has the transient ability to form a covalent ester bond with a variety of target surfaces. Once bound to target surfaces C3b becomes an essential subunit of both the classical and alternative pathway C5 cleaving enzymes.

115298678

NP_000055.2

NM_000064.3

P01024

180kDa

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. 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]

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; Inhibitor; 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 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 mg

675 USD