C4A/C4B Antibody (PACO02208)
- SKU:
- PACO02208
- Product type:
- Antibody
- Reactivity:
- Human
- Host Species:
- Rabbit
- Isotype:
- IgG
- Application:
- ELISA
- Application:
- WB
- Antibody type:
- Polyclonal
- Conjugation:
- Unconjugated
Frequently bought together:
Description
| Antibody Name: | C4A/C4B Antibody |
| Antibody SKU: | PACO02208 |
| Size: | 50ug |
| Host Species: | Rabbit |
| Tested Applications: | ELISA, WB |
| Recommended Dilutions: | WB:1:500-1:2000 |
| Species Reactivity: | Human |
| Immunogen: | synthesized peptide derived from the Internal region of human C4a/b. |
| Form: | Liquid |
| Storage Buffer: | Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide. |
| Purification Method: | The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen. |
| Clonality: | Polyclonal |
| Isotype: | IgG |
| Conjugate: | Non-conjugated |
| Synonyms: | C4A; CO4; CPAMD2; Complement C4-A; Acidic complement C4; C3 and PZP-like alpha-2-macroglobulin domain-containing protein 2; C4B; CO4; CPAMD3; Complement C4-B; Basic complement C4; C3 and PZP-like alpha-2-macroglobulin domain-containing protein 3 |
| UniProt Protein Function: | C4A: C4 plays a central role in the activation of the classical pathway of the complement system. It is processed by activated C1 which removes from the alpha chain the C4a anaphylatoxin. The remaining alpha chain fragment C4b is the major activation product and is an essential subunit of the C3 convertase (C4b2a) and the C5 convertase (C3bC4b2a) enzymes of the classical complement pathway. Defects in C4A are the cause of complement component 4A deficiency (C4AD). A rare defect of the complement classical pathway associated with the development of autoimmune disorders, mainly systemic lupus with or without associated glomerulonephritis. Defects in C4A are a cause of susceptibility to systemic lupus erythematosus (SLE). A chronic, inflammatory and often febrile multisystemic disorder of connective tissue. It affects principally the skin, joints, kidneys and serosal membranes. It is thought to represent a failure of the regulatory mechanisms of the autoimmune system. Interindividual copy- number variation (CNV) of complement component C4 and associated polymorphisms result in different susceptibilities to SLE. The risk of SLE susceptibility has been shown to be significantly increased among subjects with only two copies of total C4. A high copy number is a protective factor against SLE. |
| UniProt Protein Details: | Protein type:Inhibitor; Secreted, signal peptide; Secreted Chromosomal Location of Human Ortholog: 6p21.3 Cellular Component: extracellular region; extracellular space; plasma membrane Molecular Function:complement component C1q binding; endopeptidase inhibitor activity Biological Process: complement activation; complement activation, classical pathway; inflammatory response; innate immune response; regulation of complement activation Disease: Blood Group, Chido/rodgers System; Complement Component 4a Deficiency |
| NCBI Summary: | This gene encodes the basic form of complement factor 4, part of the classical activation pathway. The protein is expressed as a single chain precursor which is proteolytically cleaved into a trimer of alpha, beta, and gamma chains prior to secretion. The trimer provides a surface for interaction between the antigen-antibody complex and other complement components. The alpha chain may be cleaved to release C4 anaphylatoxin, a mediator of local inflammation. Deficiency of this protein is associated with systemic lupus erythematosus. This gene localizes to the major histocompatibility complex (MHC) class III region on chromosome 6. Varying haplotypes of this gene cluster exist, such that individuals may have 1, 2, or 3 copies of this gene. In addition, this gene exists as a long form and a short form due to the presence or absence of a 6.4 kb endogenous HERV-K retrovirus in intron 9. [provided by RefSeq, Jul 2008] |
| UniProt Code: | P0C0L4 |
| NCBI GenInfo Identifier: | 476007827 |
| NCBI Gene ID: | 721 |
| NCBI Accession: | P0C0L4.2 |
| UniProt Secondary Accession: | P0C0L4,P01028, P78445, Q13160, Q13906, A6H8M8, A6NHJ5 A7E2V2, B0QZR6, B0V2C8, B2RUT6, B7ZVZ6, |
| UniProt Related Accession: | P0C0L5 |
| Molecular Weight: | 187,704 Da |
| NCBI Full Name: | Complement C4-A |
| NCBI Synonym Full Names: | complement component 4B (Chido blood group) |
| NCBI Official Symbol: | C4B |
| NCBI Official Synonym Symbols: | CH; C4F; CO4; C4B1; C4B2; C4B3; C4B5; C4BD; C4B12; C4B_2; CPAMD3 |
| NCBI Protein Information: | complement C4-B |
| UniProt Protein Name: | Complement C4-A |
| UniProt Synonym Protein Names: | Acidic complement C4; C3 and PZP-like alpha-2-macroglobulin domain-containing protein 2 |
| Protein Family: | Complement C4 |
| UniProt Gene Name: | C4A |
| UniProt Entry Name: | CO4A_HUMAN |
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