BMPER Antibody (PACO58344)
- SKU:
- PACO58344
- Product Type:
- Antibody
- Reactivity:
- Human
- Host Species:
- Rabbit
- Isotype:
- IgG
- Applications:
- ELISA
- Applications:
- IHC
- Antibody Type:
- Polyclonal Antibody
- Conjugation:
- Unconjugated
Description
| Antibody Name: | BMPER Antibody (PACO58344) |
| Antibody SKU: | PACO58344 |
| Size: | 50ug |
| Host Species: | Rabbit |
| Tested Applications: | ELISA, IHC |
| Recommended Dilutions: | ELISA:1:2000-1:10000, IHC:1:200-1:500 |
| Species Reactivity: | Human |
| Immunogen: | Recombinant Human BMP-binding endothelial regulator protein (271-418AA) |
| Form: | Liquid |
| Storage Buffer: | Preservative: 0.03% Proclin 300 Constituents: 50% Glycerol, 0.01M PBS, pH 7.4 |
| Purification Method: | >95%, Protein G purified |
| Clonality: | Polyclonal |
| Isotype: | IgG |
| Conjugate: | Non-conjugated |
 | IHC image of PACO58344 diluted at 1:200 and staining in paraffin-embedded human pancreatic cancer performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a biotinylated secondary antibody and visualized using an HRP conjugated SP system. |
| Background: | Inhibitor of bone morphogenetic protein (BMP) function, it may regulate BMP responsiveness of osteoblasts and chondrocytes. |
| Synonyms: | BMP-binding endothelial regulator protein (Bone morphogenetic protein-binding endothelial cell precursor-derived regulator) (Protein crossveinless-2) (hCV2), BMPER, KIAA1965 |
| UniProt Protein Function: | BMPER: Inhibitor of bone morphogenetic protein (BMP) function, it may regulate BMP responsiveness of osteoblasts and chondrocytes. Defects in BMPER are the cause of diaphanospondylodysostosis (DSD). A rare, recessively inherited, perinatal lethal skeletal disorder. The primary skeletal characteristics of the phenotype include a small chest, abnormal vertebral segmentation, and posterior rib gaps containing incompletely differentiated mesenchymal tissue. Consistent craniofacial features include ocular hypertelorism, epicanthal folds, a depressed nasal bridge with a short nose, and low-set ears. The most commonly described extraskeletal finding is nephroblastomatosis with cystic kidneys, but other visceral findings have been described in some cases.Protein type: Secreted, signal peptide; Inhibitor; SecretedChromosomal Location of Human Ortholog: 7p14.3Cellular Component: extracellular spaceBiological Process: blood vessel endothelial cell proliferation during sprouting angiogenesis; endothelial cell activation; negative regulation of BMP signaling pathwayDisease: Diaphanospondylodysostosis |
| UniProt Protein Details: | |
| NCBI Summary: | This gene encodes a secreted protein that interacts with, and inhibits bone morphogenetic protein (BMP) function. It has been shown to inhibit BMP2- and BMP4-dependent osteoblast differentiation and BMP-dependent differentiation of the chondrogenic cells. Mutations in this gene are associated with a lethal skeletal disorder, diaphanospondylodysostosis. [provided by RefSeq, Dec 2011] |
| UniProt Code: | Q8N8U9 |
| NCBI GenInfo Identifier: | 116241270 |
| NCBI Gene ID: | 168667 |
| NCBI Accession: | Q8N8U9.3 |
| UniProt Secondary Accession: | Q8N8U9,Q8TF36, A8K1P8 |
| UniProt Related Accession: | Q8N8U9 |
| Molecular Weight: | 75,997 Da |
| NCBI Full Name: | BMP-binding endothelial regulator protein |
| NCBI Synonym Full Names: | BMP binding endothelial regulator |
| NCBI Official Symbol: | BMPER |
| NCBI Official Synonym Symbols: | CV2; CV-2; CRIM3 |
| NCBI Protein Information: | BMP-binding endothelial regulator protein |
| UniProt Protein Name: | BMP-binding endothelial regulator protein |
| UniProt Synonym Protein Names: | Bone morphogenetic protein-binding endothelial cell precursor-derived regulator; Protein crossveinless-2; hCV2 |
| Protein Family: | BMP-binding endothelial regulator protein |
| UniProt Gene Name: | BMPER |
| UniProt Entry Name: | BMPER_HUMAN |
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