抗体

山羊抗人类C3

MBS316106

10毫升

175美元

多克隆

山羊

未共轭

抗体

山羊抗人类C3

补体C3

Goat Antibody to Human Complement Component 3 (C3)

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

多克隆

山羊

1663

Complement C3. Monospecific by immunoelectrophoresis (IEP) against normal human plasma and 2x concentrated normal human serum.

Delipidation followed by solid phase adsorption. Product is 0.2um filtered.

Monospecific, Liquid

Total protein: 53 OD280nm Units/ml

Store at 2 to 8 degree C. Slight precipitation may occur on storage which can be removed by centrifugation and should not affect performance characteristics.

TIA

Immunogen: Purified human C3. Grade: Turbidimetric grade.

QC Information: +/-10% of master reference lot by Hitachi 704 system. Buffer: 0.05M Tris, 0.5M Sodium chloride, pH 7.5. Preservative: 0.05% Sodium azide. Warnings: This product contains sodium azide, which has been classified as Xn (Harmful) in European Directive 67/548/EEC in the concentration range of 0.1 - 1.0 %. When disposing of this reagent through lead or copper plumbing, flush with copious volumes of water to prevent azide build-up in drains.

Polyclonal Antibodies to Proteins and Bioactive Peptides

119370332

P01024.2

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]

Function: 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.Derived from proteolytic degradation of complement C3, C3a anaphylatoxin is a mediator of local inflammatory process. It induces the contraction of smooth muscle, increases vascular permeability and causes histamine release from mast cells and basophilic leukocytes. Subunit structure: C3 precursor is first processed by the removal of 4 Arg residues, forming two chains, beta and alpha, linked by a disulfide bond. C3 convertase activates C3 by cleaving the alpha chain, releasing C3a anaphylatoxin and generating C3b (beta chain + alpha' chain). C3dg interacts with CR2 (via the N-terminal Sushi domains 1 and 2). During pregnancy, C3dg exists as a complex (probably a 2:2:2 heterohexamer) with AGT and the proform of PRG2. Interacts with VSIG4. C3b interacts with herpes simplex virus 1 (HHV-1) and herpes simplex virus 2 (HHV-2) envelope glycoprotein C; this interaction inhibits the activation of the complement system. Interacts with S.aureus immunoglobulin-binding protein sbi, this prevents interaction between C3dg and CR2. Interacts with S.aureus fib. Ref.6 Ref.9 Ref.18 Ref.19 Ref.29 Ref.30. Subcellular location: Secreted. Tissue specificity: Plasma. Ref.17. Post-translational modification: C3b is rapidly split in two positions by factor I and a cofactor to form iC3b (inactivated C3b) and C3f which is released. Then iC3b is slowly cleaved (possibly by factor I) to form C3c (beta chain + alpha' chain fragment 1 + alpha' chain fragment 2), C3dg and C3f. Other proteases produce other fragments such as C3d or C3g.Phosphorylation sites are present in the extracelllular medium. Polymorphism: There are two alleles: C3S (C3 slow), the most common allele in all races and C3F (C3 fast), relatively frequent in Caucasoids, less common in Black Americans, extremely rare in Orientals. Involvement in disease: Defects in C3 are the cause of complement component 3 deficiency (C3D) [. MIM:613779]. 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. Ref.21 Ref.22 Ref.23 Ref.34 Ref.35Genetic variation in C3 is associated with susceptibility to age-related macular degeneration type 9 (ARMD9) [. MIM:611378]. 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. Ref.36Defects in C3 are a cause of susceptibility to hemolytic uremic syndrome atypical type 5 (AHUS5) [. MIM:612925]. 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. Note=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. Ref.37 Ref.38. Sequence similarities: Contains 1 anaphylatoxin-like domain.Contains 1 NTR domain. Caution: According to Ref.30, the interaction surface between C3 and CR2 reported in Ref.22 is artifactual and can be ascribed to the presence of zinc acetate in the buffer.

10毫升

175美元