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Product Name Anti-BMPR1A/RBITC Chinese Name 罗丹明(RBITC)标记的骨成型蛋白受体1A抗体 Alias BMPR-1A; Activin A receptor type II like kinase 3; Activin receptor like kinase 3; ACVRLK 3; ACVRLK3; ALK 3; ALK3; BMPR 1A; Bmpr; Bone morphogenetic protein receptor type IA; Bone morphogenetic protein receptor type IA precursor; BR 1a; BR1a; CD 292; CD292; CD292 antigen; EC 2.7.11.30; Serine threonine protein kinase receptor R5; Serine threonine protein kinase receptor R5 precursor; SKR 5; SKR5; zBMPR IA; zBMPRIA. Research Area Tumour Cardiovascular Cell biology Signal transduction Stem cells Growth factors and hormones transcriptional regulatory factor Kinases and Phosphatases The cell membrane受体 Cell Surface Molecule Immunogen Species Rabbit Clonality Polyclonal React Species Mouse, Rat, (predicted: Human, ) Applications IF=1:50-200
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.Molecular weight 60kDa Form Lyophilized or Liquid Concentration 1mg/ml immunogen KLH conjugated synthetic peptide derived from human BMPR-1A Lsotype IgG Purification affinity purified by Protein A Storage Buffer 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. Storage Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C. Product Detail background:
The bone morphogenetic protein (BMP) receptors are a family of transmembrane serine/threonine kinases that include the type I receptors BMPR1A and BMPR1B and the type II receptor BMPR2. These receptors are also closely related to the activin receptors, ACVR1 and ACVR2. The ligands of these receptors are members of the TGF-beta superfamily. TGF-betas and activins transduce their signals through the formation of heteromeric complexes with 2 different types of serine (threonine) kinase receptors: type I receptors of about 50-55 kD and type II receptors of about 70-80 kD. Type II receptors bind ligands in the absence of type I receptors, but they require their respective type I receptors for signaling, whereas type I receptors require their respective type II receptors for ligand binding. [provided by RefSeq].
Function:
On ligand binding, forms a receptor complex consisting of two type II and two type I transmembrane serine/threonine kinases. Type II receptors phosphorylate and activate type I receptors which autophosphorylate, then bind and activate SMAD transcriptional regulators. Receptor for BMP-2 and BMP-4.
Subcellular Location:
Membrane; Single-pass type I membrane protein.
Tissue Specificity:
Highly expressed in skeletal muscle.
DISEASE:
Defects in BMPR1A are a cause of juvenile polyposis syndrome (JPS) [MIM:174900]; also known as juvenile intestinal polyposis (JIP). JPS is an autosomal dominant gastrointestinal hamartomatous polyposis syndrome in which patients are at risk for developing gastrointestinal cancers. The lesions are typified by a smooth histological appearance, predominant stroma, cystic spaces and lack of a smooth muscle core. Multiple juvenile polyps usually occur in a number of Mendelian disorders. Sometimes, these polyps occur without associated features as in JPS; here, polyps tend to occur in the large bowel and are associated with an increased risk of colon and other gastrointestinal cancers.
Defects in BMPR1A are a cause of Cowden disease (CD) [MIM:158350]. CD is an autosomal dominant cancer syndrome characterized by multiple hamartomas and by a high risk for breast, thyroid and endometrial cancers.
Similarity:
Belongs to the protein kinase superfamily. TKL Ser/Thr protein kinase family. TGFB receptor subfamily.
Contains 1 GS domain.
Contains 1 protein kinase domain.
Database links:Entrez Gene: 657 Human
Entrez Gene: 12166 Mouse
Omim: 601299 Human
SwissProt: P36894 Human
SwissProt: P36895 Mouse
Unigene: 524477 Human
Unigene: 237825 Mouse
Unigene: 88925 Rat
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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