antibody

Mouse Anti-Human ALK6 (BMP R1B)

MBS690053

0.1 mg

490 USD

monoclonal

mouse

BMP

[3E12]

western blot, immunohistochemistry

Antibody

Mouse Anti-Human ALK6 (BMP R1B)

[ALK6 (BMP R1B)]

[BMPR1B; ALK6; ALK-6, CDw293]

[bone morphogenetic protein receptor type-1B isoform b; Bone morphogenetic protein receptor type-1B; bone morphogenetic protein receptor type-1B; BMPR-1B; BMP type-1B receptor; activin receptor-like kinase 6; serine/threonine receptor kinase; bone morphogenetic protein receptor, type IB]

[ALK6 (BMP R1B)]

[BMPR1B; BMPR1B; ALK6; ALK-6; CDw293; BMP type-1B receptor; BMPR-1B]

BMR1B_HUMAN

Monoclonal

IgG2

[3E12]

Mouse

Human

502

This antibody was selected for its ability to detect human ALK6. No cross reactivity was found to ALK3 (BMPR-IA).

Protein G affinity chromatography

Lyophilized

Lyophilized samples are stable for 2 years from date of receipt when stored at -70 degree C. Reconstituted antibody can be aliquoted and stored frozen at < -20 degree C for at least for six months without detectable loss of activity. Shipping: Ships at RT or 4 degree C.

Western Blot (WB), Immunohistochemistry (IHC)

WB: 1:500-1:1000. IHC: 1:50-1:200

IHC

Antibody Generation: This antibody was produced from a hybridoma (mouse myeloma fused with spleen cells from a mouse immunized with recombinant human ALK6 extra cellular domain. IgG2 fraction of the tissue culture supernatant was purified by Protein G affinity chromatography. Antigen: Recombinant human ALK6 extracellular domain.

Reconstitution: Reconstitute the antibody with 200 ul sterile PBS and the final concentration is 500 ug/ml. Reconstruction Buffer: PBS (sterile)

Cellular responses to bone morphogenetic proteins (BMPs) have been shown to be mediated by the formation of heterooligomeric complexes of the type I and type II serine/threonine kinase receptors. BMP receptor IB (BMPRIB), also known as activin receptor-like kinase (ALK)6, is one of seven known type I serine/threonine kinases that are required for the signal transduction of TGFbeta family cytokines. In contrast to the TGFbeta receptor system in which the type I receptor does not bind TGFbeta in the absence of the type II receptor, type I receptors involved in BMP signaling (including BMPRIA, BMPRIB/ ALK6, and ActRI/ ALK2) can independently bind the various BMP family proteins in the absence of type II receptors. Recombinant soluble BMPRIB binds BMP4 with high-affinity in solution and is a potent BMP4 antagonist in vitro. BMPRIB is expressed in various tissues during embryogenesis. In adult tissues, BMPRIB is only found in the brain. The extracellular domain of BMPRIB shares little amino acid sequence identity with the other mammalian ALK type I receptor kinases, but the cysteine residues are conserved. Human and mouse BMPRIB are highly conserved and share 98% amino acid sequence identity.

4502431

NP_001194

NM_001203.2

O00238

BMP Signalling And Regulation Pathway (198910); Cytokine-cytokine Receptor Interaction Pathway (83051); Cytokine-cytokine Receptor Interaction Pathway (460); Hippo Signaling Pathway (749777); Hippo Signaling Pathway (750388); Ovarian Infertility Genes Pathway (198801); Signal Transduction Pathway (477114); Signaling By BMP Pathway (106336); TGF-beta Signaling Pathway (83064); TGF-beta Signaling Pathway (475)

This gene encodes a member of the bone morphogenetic protein (BMP) receptor family of transmembrane serine/threonine kinases. The ligands of this receptor are BMPs, which are members of the TGF-beta superfamily. BMPs are involved in endochondral bone formation and embryogenesis. These proteins transduce their signals through the formation of heteromeric complexes of 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. Mutations in this gene have been associated with primary pulmonary hypertension. Several transcript variants encoding two different isoforms have been found for this gene. [provided by RefSeq, Feb 2012]

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 BMP7/OP-1 and GDF5. Catalytic activity: ATP + [receptor-protein] = ADP + [receptor-protein] phosphate. Cofactor: Magnesium or manganese . By similarity. Subcellular location: Membrane; Single-pass type I membrane protein. Involvement in disease: Acromesomelic chondrodysplasia, with genital anomalies (AMDGA) [MIM:609441]: A form of chondrodysplasia. Acromesomelic chondrodysplasias are rare hereditary skeletal disorders characterized by short stature, very short limbs and hand/foot malformations. The severity of limb abnormalities increases from proximal to distal with profoundly affected hands and feet showing brachydactyly and/or rudimentary fingers (knob-like fingers).Note: The disease is caused by mutations affecting the gene represented in this entry. Ref.8Brachydactyly A2 (BDA2) [MIM:112600]: A form of brachydactyly. Brachydactyly defines a group of inherited malformations characterized by shortening of the digits due to abnormal development of the phalanges and/or the metacarpals. In brachydactyly type A2 shortening of the middle phalanges is confined to the index finger and the second toe, all other digits being more or less normal. Because of a rhomboid or triangular shape of the affected middle phalanx, the end of the second finger usually deviates radially.Note: The disease is caused by mutations affecting the gene represented in this entry. Ref.7. Sequence similarities: Belongs to the protein kinase superfamily. TKL Ser/Thr protein kinase family. TGFB receptor subfamily.Contains 1 GS domain.Contains 1 protein kinase domain.

0.1 mg

490 USD