GOAT ANTI HUMAN C3

GWB-BBDEC5

Polyclonal IgG

Recognises human C3 a secreted protein that plays a central role in the activation of the complement system. This reagent is available in bulk quantities and may be made available in other formats. Please enquire for further details. Recommended Secondary Antibodies: Rabbit Anti Goat IgG (Fc)

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 diseaseDefects in C3 are the cause of complement component 3 deficiency (C3D) [MIM:120700]. 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. 18Ref. 19Ref. 20Ref. 27Ref. 28Genetic 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. 29Defects 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. 30Sequence similaritiesContains 1 anaphylatoxin-like domain. Contains 1 NTR domain. 1. Haruta Y. and Seon B. K. (1986) Distinct human leukemia-associated cell surface glycoprotein GP160 defined by monoclonal antibody SN6. Proc. Natl. Acad. Sci. USA 83: 7898-7902. 2. Jin H. J. et al. (2010) GD2expression is closely associated with neuronal differentiation of human umbilical cord blood-derived mesenchymal stem cells. Cell Mol Life Sci. Feb 18. [Epub ahead of print] [1] \" Identification and expression of two forms of the human transforming growth factor-beta-binding protein endoglin with distinct cytoplasmic regions. \" Bellon T. Corbi A. Lastres P. Cales C. Cebrian M. Vera S. Cheifetz S. Massague J. Letarte M. Bernabeu C. Eur. J. Immunol. 23:2340-2345(1993) [PubMed: 8370410] [Abstract]Cited for: NUCLEOTIDE SEQUENCE [MRNA] (ISOFORM SHORT). [2] \" DNA sequence and analysis of human chromosome 9. \" Humphray S. J. Oliver K. Hunt A. R. Plumb R. W. Loveland J. E. Howe K. L. Andrews T. D. Searle S. Hunt S. E. Scott C. E. Jones M. C. Ainscough R. Almeida J. P. Ambrose K. D. Ashwell R. I. S. Babbage A. K. Babbage S. Bagguley C. L. Bailey J. Banerjee R. Barker D. J. Barlow K. F. Bates K. Beasley H. Beasley O. Bird C. P. Bray-Allen S. Brown A. J. Brown J. Y. Burford D. Burrill W. Burton J. Carder C. Carter N. P. Chapman J. C. Chen Y. Clarke G. Clark S. Y. Clee C. M. Clegg S. Collier R. E. Corby N. Crosier M. Cummings A. T. Davies J. Dhami P. Dunn M. Dutta I. Dyer L. W. Earthrowl M. E. Faulkner L. Fleming C. J. Frankish A. Frankland J. A. French L. Fricker D. G. Garner P. Garnett J. Ghori J. Gilbert J. G. R. Glison C. Grafham D. V. Gribble S. Griffiths C. Griffiths-Jones S. Grocock R. Guy J. Hall R. E. Hammond S. Harley J. L. Harrison E. S. I. Hart E. A. Heath P. D. Henderson C. D. Hopkins B. L. Howard P. J. Howden P. J. Huckle E. Johnson C. Johnson D. Joy A. A. Kay M. Keenan S. Kershaw J. K. Kimberley A. M. King A. Knights A. Laird G. K. Langford C. Lawlor S. Leongamornlert D. A. Leversha M. Lloyd C. Lloyd D. M. Lovell J. Martin S. Mashreghi-Mohammadi M. Matthews L. McLaren S. McLay K. E. McMurray A. Milne S. Nickerson T. Nisbett J. Nordsiek G. Pearce A. V. Peck A. I. Porter K. M. Pandian R. Pelan S. Phillimore B. Povey S. Ramsey Y. Rand V. Scharfe M. Sehra H. K. Shownkeen R. Sims S. K. Skuce C. D. Smith M. Steward C. A. Swarbreck D. Sycamore N. Tester J. Thorpe A. Tracey A. Tromans A. Thomas D. W. Wall M. Wallis J. M. West A. P. Whitehead S. L. Willey D. L. Williams S. A. Wilming L. Wray P. W. Young L. Ashurst J. L. Coulson A. Blocker H. Durbin R. M. Sulston J. E. Hubbard T. Jackson M. J. Bentley D. R. Beck S. Rogers J. Dunham I. Nature 429:369-374(2004) [PubMed: 15164053] [Abstract]Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA]. [3] Mural R. J. Istrail S. Sutton G. G. Florea L. Halpern A. L. Mobarry C. M. Lippert R. Walenz B. Shatkay H. Dew I. Miller J. R. Flanigan M. J. Edwards N. J. Bolanos R. Fasulo D. Halldorsson B. V. Hannenhalli S. Turner R. Yooseph S. Lu F. Nusskern D. R. Shue B. C. Zheng X. H. Zhong F. Delcher A. L. Huson D. H. Kravitz S. A. Mouchard L. Reinert K. Remington K. A. Clark A. G. Waterman M. S. Eichler E. E. Adams M. D. Hunkapiller M. W. Myers E. W. Venter J. C. Submitted (JUL-2005) to the EMBL/GenBank/DDBJ databasesCited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA]. [4] \" Primary structure of endoglin an RGD-containing glycoprotein of human endothelial cells. \" Gougos A. Letarte M. J. Biol. Chem. 265:8361-8364(1990) [PubMed: 1692830] [Abstract]Cited for: NUCLEOTIDE SEQUENCE [GENOMIC DNA / MRNA] OF 14-658 PROTEIN SEQUENCE OF 26-36 (ISOFORM LONG). Tissue: Umbilical vein. [5] \" Endoglin a TGF-beta binding protein of endothelial cells is the gene for hereditary haemorrhagic telangiectasia type 1. \" McAllister K. A. Grogg K. M. Johnson D. W. Gallione C. J. Baldwin M. A. Jackson C. E. Helmbold E. A. Markel D. S. McKinnon W. C. Murrell J. McCormick M. K. Pericak-Vance M. A. Heutink P. Oostra B. A. Haitjema T. Westerman C. J. Porteous M. E. Guttmacher A. E. Letarte M. Marchuk D. A. Nat. Genet. 8:345-351(1994) [PubMed: 7894484] [Abstract]Cited for: NUCLEOTIDE SEQUENCE [GENOMIC DNA] OF 122-378. [6] \" Identification of Tctex2beta a novel dynein light chain family member that interacts with different transforming growth factor-beta receptors. \" Meng Q. -J. Lux A. Holloschi A. Li J. Hughes J. M. X. Foerg T. McCarthy J. E. G. Heagerty A. M. Kioschis P. Hafner M. Garland J. M. J. Biol. Chem. 281:37069-37080(2006) [PubMed: 16982625] [Abstract]Cited for: INTERACTION WITH TCTEX1D4. [7] \" The interaction of endoglin with beta-arrestin2 regulates transforming growth factor-beta-mediated ERK activation and migration in endothelial cells. \" Lee N. Y. Blobe G. C. J. Biol. Chem. 282:21507-21517(2007) [PubMed: 17540773] [Abstract]Cited for: INTERACTION WITH ARRB2. [8] \" Glycoproteomics analysis of human liver tissue by combination of multiple enzyme digestion and hydrazide chemistry. \" Chen R. Jiang X. Sun D. Han G. Wang F. Ye M. Wang L. Zou H. J. Proteome Res. 8:651-661(2009) [PubMed: 19159218] [Abstract]Cited for: GLYCOSYLATION [LARGE SCALE ANALYSIS] AT ASN-134 MASS SPECTROMETRY. Tissue: Liver. [9] \" Characterization of endoglin and identification of novel mutations in hereditary hemorrhagic telangiectasia. \" Shovlin C. L. Hughes J. M. B. Scott J. Seidman C. E. Seidman J. G. Am. J. Hum. Genet. 61:68-79(1997) [PubMed: 9245986] [Abstract]Cited for: VARIANT HHT1 192-ARG--PRO-198 DEL VARIANT MET-5. [10] \" A novel missense mutation in the endoglin gene in hereditary hemorrhagic telangiectasia. \" Yamaguchi H. Azuma H. Shigekiyo T. Inoue H. Saito S. Thromb. Haemost. 77:243-247(1997) [PubMed: 9157574] [Abstract]Cited for: VARIANT HHT1 ASP-160.