Antibody (Suitable for clinical applications)
Specification | Recommendation |
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Recommended Dilution (Conc) | 1:50-1:100 |
Pretreatment | EDTA Buffer pH 8.0 |
Incubation Parameters | 30 min at Room Temperature |
Prior to use, inspect vial for the presence of any precipitate or other unusual physical properties. These can indicate that the antibody has degraded and is no longer suitable for patient samples. Please run positive and negative controls simultaneously with all patient samples to account and control for errors in laboratory procedure. Use of methods or materials not recommended by enQuire Bio including change to dilution range and detection system should be routinely validated by the user.
C4d Information for Pathologists
Summary:
C4d is a component of the classical and lectin complement cascade. C4d is a cleavage product of C4 which is first cleaved into C4a and C4b which is furter processed to C4c and C4d. C4d binds close to C4d and its presence is stable making it a good biomarker for complement activation.
Common Uses By Pathologists:
C4d has been classically used to identify sites of tissue injury mediated by anti-donor antibodies in transplant biology. Limitations in this application exist. For instance, in ABO incompatibility, C4d is a marker of donor accomodation vs the more virulent donor rejection classically ascribed to C4d staining. In patients with certain systemic autoimmune diseases and in pregnancy, C4d staining is also observed.
C4d General Information | |
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Alternate Names | |
Molecular Weight | |
192.8 kDa | |
Chromosomal Location | |
[chr: CHR_HSCHR6_MHC_MCF_CTG1] [chr_start: 32091205] [chr_end: 32111862] [strand: 1]; [chr: CHR_HSCHR6_MHC_QBL_CTG1] [chr_start: 31972402] [chr_end: 31993060] [strand: 1]; p21.33 [chr: 6] [chr_start: 31982057] [chr_end: 32002681] [strand: 1]; [chr: CHR_HSCHR6_MHC_DBB_CTG1] [chr_start: 31964204] [chr_end: 31984862] [strand: 1]; p21.33 [chr: 6] [chr_start: 32014795] [chr_end: 32035418] [strand: 1]; [chr: CHR_HSCHR6_MHC_MCF_CTG1] [chr_start: 32058468] [chr_end: 32058494] [strand: 1]; [chr: CHR_HSCHR6_MHC_SSTO_CTG1] [chr_start: 32007416] [chr_end: 32028073] [strand: 1]; [chr: CHR_HSCHR6_MHC_COX_CTG1] [chr_start: 31969570] [chr_end: 31983859] [strand: 1]; [chr: CHR_HSCHR6_MHC_DBB_CTG1] [chr_start: 31996944] [chr_end: 32011233] [strand: 1] | |
Curated Database and Bioinformatic Data | |
Gene Symbol | C4A |
Entrez Gene ID | 720; 721 |
RefSeq Protein Accession(s) | NP_009224; NP_001239133; NP_001002029 |
RefSeq mRNA Accession(s) | NM_007293; NM_001252204; NM_001002029 |
RefSeq Genomic Accession(s) | NC_000006; NT_167248; NG_011638; NT_167245; NG_011639; NT_113891; NT_167249 |
UniProt ID(s) | P0C0L4 |
PharmGKB ID(s) | PA25904; PA25903 |
KEGG Gene ID(s) | hsa:720; hsa:721 |
Associated Diseases (KEGG IDs) | Complement component 4A deficiency (C4AD) [MIM:614380]: A rare defect of the complement classical pathway associated with the development of autoimmune disorders, mainly systemic lupus with or without associated glomerulonephritis. {ECO:0000269|PubMed:8473511}. The disease is caused by mutations affecting the gene represented in this entry.; Systemic lupus erythematosus (SLE) [MIM:152700]: A chronic, relapsing, inflammatory, and often febrile multisystemic disorder of connective tissue, characterized principally by involvement of the skin, joints, kidneys and serosal membranes. It is of unknown etiology, but is thought to represent a failure of the regulatory mechanisms of the autoimmune system. The disease is marked by a wide range of system dysfunctions, an elevated erythrocyte sedimentation rate, and the formation of LE cells in the blood or bone marrow. {ECO:0000269|PubMed:10092831, ECO:0000269|PubMed:17503323}. Disease susceptibility is associated with variations affecting the gene represented in this entry. 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. |
General Description of C4d . | |
Complement 4d (C4d) is the most clinically used marker for humoral rejection. It is a degradation product of the activated complement factor C4b. Complement 4d is typically initiated by binding of antibodies to specific target molecules. Following activation and degradation of the C4 molecule, thio-ester groups are exposed, which allow transient, covalent binding of the degradation product Complement 4d to endothelial cell surfaces and extracellular matrix components of vascular basement membranes near the sites of C4 activation. Complement 4d is also found in intracytoplasmic vacuoles of endothelial cells. Covalent binding renders C4d a stable molecule that can easily be detected by immunohistochemistry. |
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