SKU: WR4069 Categories: ,

U-Blot® CD105 Rabbit mAb

Price range: $268.00 through $328.00

SKU: WR4069-50
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Product details

Background:This gene encodes a homodimeric transmembrane protein which is a major glycoprotein of the vascular endothelium. This protein is a component of the transforming growth factor beta receptor complex and it binds to the beta1 and beta3 peptides with high affinity. Mutations in this gene cause hereditary hemorrhagic telangiectasia, also known as Osler-Rendu-Weber syndrome 1, an autosomal dominant multisystemic vascular dysplasia. This gene may also be involved in preeclampsia and several types of cancer. Alternatively spliced transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, May 2013],

Specifications

Size50μL/100μL
TargetCD105(Endoglin)
ReactivityHuman
ApplicationsWB, IHC, IF, IP, ELISA
MW(Calculated)70kD
MW(Observed)95kD
Host SpeciesRabbit
IsotypeIgG,Kappa
Conjugate/ModificationUnmodified
Recommended Dilution RatioIHC 1:100-200; WB 1:1000-5000; IF 1:200-1000; ELISA 1:5000-20000; IP 1:50-200 / Note: For IHC, we suggest antigen retrieval with TE buffer pH 9.0 (Cat#WT1004)
FormulationPBS, 50% glycerol, 0.05% Proclin 300, 0.05%BSA
PurificationRecombinant Antibody expressed in animal component-free (ACF) media, purified via Protein A affinity chromatography.
Storage-15°C to -25°C/1 year(Do not lower than -25°C)
Concentration
ClonalityMonoclonal
Clone NumberRM0152R
ImmunogenThe specific immunogen used to produce this antibody is proprietary information.
SpecificityEndogenous
Gene NameENG END
Protein NameEndoglin (CD antigen CD105)
Other Name
SpeciesHuman
Gene ID2022
UniprotP17813
Species.1
Gene ID.1
Uniprot.1
Species.2
Gene ID.2
Uniprot.2
Cellular LocalizationCytoplasm
Tissue ExpressionDetected on umbilical veil endothelial cells (PubMed:10625079). Detected in placenta (at protein level) (PubMed:1692830). Detected on endothelial cells (PubMed:1692830).
Signaling_pathway
Research Areas
FunctionDisease:Defects in ENG are the cause of hereditary hemorrhagic telangiectasia type 1 (HHT1) [MIM:187300, 108010]; also known as Osler-Rendu-Weber syndrome 1 (ORW1). HHT1 is an autosomal dominant multisystemic vascular dysplasia, characterized by recurrent epistaxis, muco-cutaneous telangiectases, gastro-intestinal hemorrhage, and pulmonary (PAVM), cerebral (CAVM) and hepatic arteriovenous malformations; all secondary manifestations of the underlying vascular dysplasia. Although the first symptom of HHT1 in children is generally nose bleed, there is an important clinical heterogeneity.,Function:Major glycoprotein of vascular endothelium. May play a critical role in the binding of endothelial cells to integrins and/or other RGD receptors.,subunit:Homodimer that forms an heteromeric complex with the signaling receptors for transforming growth factor-beta: TGF-beta receptors I and/or II. It is able to bind TGF-beta 1, and 3 efficiently and TGF-beta 2 less efficiently. Interacts with TCTEX1D4.,tissue specificity:Endoglin is restricted to endothelial cells in all tissues except bone marrow.,
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