Complement C3 Native Protein | C3 native protein

Purified native Human Complement C3 protein

Cat. Num. AAA5308298

• Gene Names: C3; ASP; C3a; C3b; AHUS5; ARMD9; CPAMD1; HEL-S-62p
• Purity: >99% pure
• Synonyms: Complement C3; Purified native Human Complement C3 protein; C3 protein; Complement C-3; Complement C 3; Complement C-3 protein; Complement C 3 protein; C3 native protein

• Host: Human Plasma
• Purity/Purification: >99% pure
• Form/Format: Frozen liquid in Sodium Phosphate Buffer, pH 7.2 with 150mM NaCl.
• Concentration: 1.67mg/ml (varies by lot)
• Sequence Length: 1663

• Species: Human
• Protein Type: Native
• Contaminants: Shows only one band to C3 molecular weight; ~180KD
• Biohazard: Non-reactive for HIV-1/HCV/HBV by NAT, HBsAg, HCV Ab, HIV 1 & 2 Ab, and RPR by currently approved FDA methods. However, handle as though potentially infectious.
• Biological Significance: Native human C3 is a naturally glycosylated polypeptide containing two disulfide-linked chains. C3 is central to the activation of all three pathways of complement activation. Initiation of each pathway generates proteolytic enzyme complexes (C3 convertases) which are bound to the target surface. These enzymes cleave a peptide bond in C3 releasing the anaphylatoxin C3a and activating C3b. For a brief time (~60 us) this nascent C3b is capable of reacting with and covalently coupling to hydroxyl groups on the target surface.
• Dry Ice Shipment: Extra charge may be applied to your shipping cost as dry ice is required to ship this product.
• Preparation and Storage: Ships on dry ice. Upon receipt, store at -20°C.

Product Categories/Family for C3 native protein

Immunology

NCBI and Uniprot Product Information

• NCBI GI #: 115298678
• NCBI GeneID: 718
• NCBI Accession #: NP_000055.2
• NCBI GenBank Nucleotide #: NM_000064.3
• UniProt Accession #: P01024; A7E236
• NCBI Official Full Name: complement C3 preproprotein
• NCBI Official Synonym Full Names: complement C3
• NCBI Official Symbol: C3
• NCBI Official Synonym Symbols: ASP; C3a; C3b; AHUS5; ARMD9; CPAMD1; HEL-S-62p
• NCBI Protein Information: complement C3
• UniProt Protein Name: Complement C3
• Protein Family: Complement C3
• UniProt Gene Name: C3
• UniProt Synonym Gene Names: CPAMD1; C3bc; ASP

NCBI Description

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]

Uniprot Description

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

Cellular Component: extracellular region; extracellular space; plasma membrane

Molecular Function: C5L2 anaphylatoxin chemotactic receptor binding; protein binding; receptor binding; serine-type endopeptidase activity

Biological Process: complement activation; complement activation, alternative pathway; G-protein coupled receptor protein signaling pathway; immune response; positive regulation of G-protein coupled receptor protein signaling pathway; positive regulation of protein amino acid phosphorylation; regulation of complement activation; regulation of immune response; signal transduction

Disease: Complement Component 3 Deficiency, Autosomal Recessive; Hemolytic Uremic Syndrome, Atypical, Susceptibility To, 5; Macular Degeneration, Age-related, 9