catalog number :
MBS537683
products type :
Native Protein
products full name :
Complement C4a protein
products short name :
Complement C4a
products name syn :
C4a protein
other names :
complement C4-A isoform 1 preproprotein; Complement C4-A; complement C4-A; complement component 4A (Rodgers blood group); Acidic complement C4; C3 and PZP-like alpha-2-macroglobulin domain-containing protein 2
other gene names :
C4A; C4A; C4; RG; C4S; CO4; C4A2; C4A3; C4A4; C4A6; C4AD; CPAMD2; CO4; CPAMD2
uniprot entry name :
CO4A_HUMAN
form :
10mM Hepes, pH 7.0, with 120mM NaCl, no preservatives added.
storage stability :
Aliquot and store at -70 degree C or lower. Avoid repeated freeze/thaw cycles.
other info1 :
Source Note: Human serum/plasma. Protein Type: Native. Biological Significance: C4a is a member of the anaphylatoxin family of three proteins (C3a, C4a and C5a) produced by the activation of complement. It is an unglycosylated polypeptide containing 77 amino acids with a molecular mass of 8,759 daltons.
other info2 :
Biohazard Information: The source of C4a is human serum, therefore appropriate precautions must be observed even though the source was shown by certified tests to be negative for HBsAg, HTLV-I/II, STS, and for antibodies to HCV, HIV-1 and HIV-II. Injection can cause anaphylatic shock which is a generalized circulatory collapse similar to that caused by an allergic reaction. Dry Ice Shipment: Extra charge fee may add to your shipping cost as dry ice is required to ship this product.
products categories :
Immunology; Native Protein; Complements
products description :
Purified Complement C4a protein isolated from human plasma
ncbi acc num :
NP_009224.2
ncbi gb acc num :
NM_007293.2
ncbi mol weight :
8.75 kDa
ncbi pathways :
Activation Of C3 And C5 Pathway (106412); Allograft Rejection Pathway (920963); Complement Activation, Classical Pathway (198823); Complement And Coagulation Cascades Pathway (83073); Complement And Coagulation Cascades Pathway (484); Complement Cascade Pathway (106405); Immune System Pathway (106386); Initial Triggering Of Complement Pathway (106406); Innate Immune System Pathway (106387); Pertussis Pathway (218111)
ncbi summary :
This gene encodes the acidic 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 is cleaved to release C4 anaphylatoxin, an antimicrobial peptide and a mediator of local inflammation. Deficiency of this protein is associated with systemic lupus erythematosus and type I diabetes mellitus. 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. Two transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Nov 2014]
uniprot summary :
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. Protein type: Secreted; Inhibitor; Secreted, signal peptide. Chromosomal Location of Human Ortholog: 6p21.3. Cellular Component: plasma membrane; extracellular region. Molecular Function: complement component C1q binding; endopeptidase inhibitor activity. Biological Process: regulation of complement activation; innate immune response; inflammatory response; complement activation, classical pathway; complement activation. Disease: Complement Component 4a Deficiency; Blood Group, Chido/rodgers System