Antibody data
- Antibody Data
- Antigen structure
- References [0]
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- Validations
- Western blot [1]
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- Product number
- BS60010 - Provider product page
- Provider
- Bioworld Technology, Inc
- Product name
- Endoglin polyclonal antibody
- Antibody type
- Polyclonal
- Antigen
- A synthetic peptide corresponding to residues in Human Endoglin.
- Description
- Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant disorder characterized by vascular abnormalities such as dilated vessels, hemorrhages, liver and lung congestion, and brain or heart ischemia. Mutations in two genes, Endoglin (also designated CD105) and ALK-1 (Activin receptorlike kinase 1, also designated TGFÉÇ superfamily RI), are responsible for HHT. Endoglin is mutated in HHT1, and ALK-1 is mutated in HHT2, both of which are thought to be caused by haploinsufficiency. Endoglin and ALK-1 are type III and type I members of the TGFÉÇ receptor superfamily, respectively, that are expressed on vascular endothelial cells. Endoglin can only bind ligands of the TGFÉÇ superfamily via association with the respective ligand binding receptors for TGFÉÇ1, TGFÉÇ3, Activin-A, BMP-2 and BMP-7. The human ALK-1 gene encodes two protein species which exist as a result of either glycosylation or alternative splicing events. ALK-1 preferentially binds TGFÉÇ1 and is expressed in bone marrow stromal cells, lung, brain, kidney and spleen.
- Reactivity
- Human, Mouse
- Host
- Rabbit
- Isotype
- IgG
- Vial size
- 100ul
- Concentration
- 1 mg/ml
- Storage
- Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.
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Supportive validation
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- Bioworld Technology, Inc (provider)
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- Experimental details
- Western blot (WB) analysis of Endoglin pAb at 1:500 dilutionLane1:Hela whole cell lysateLane2:A549 whole cell lysateLane3:NIH-3T3 whole cell lysate