Name: BMPER Antibody (PACO58344)
SKU: PACO58344
Availability: OutOfStock

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