The TGFβ (transforming growth factor β) superfamily proteins are pleiotropic cytokines that regulate a diverse range of cellular processes, including proliferation, differentiation, migration, adhesion and death.  The TGFβ superfamily elicits 4 signaling pathways: TGFβ, Bone Morphogenic Protein (BMP), Activin, and Nodal.  Each pathway signals through a heteromeric receptor complex composed of at least two type-I and two type-II transmembrane receptors containing cytoplasmic serine/threonine kinase domains. These receptors are distinguished by the presence of a glycine/serine-rich juxta-membrane domain found only in the type I receptors. Either type receptor may initially bind ligand, followed by the recruitment of an alternate-type receptor counterpart to form a signal-activating complex. The type I receptors are also referred to as ALKs (Activin receptor-like kinases). ACVRL1 (Activin A receptor type II-like 1), also known as ALK1, HHT2, SKR3, TSR-I, and ACVRLK1, is an endothelial-specific type I receptor in the TGFβ receptor (TGFBR) family. ACVRL1 is expressed highly in endothelial cells and other highly vascularized tissues. ACVRL1 acts as a type I receptor for TGFβ1, TGFβ3, BMP9 and BMP10 and an important regulator of normal blood vessel development or repair. It may bind Activin as well.  The expression pattern of ALK­1 parallels that of endoglin. Mutations in ACVRL1 as well as in endoglin are associated with hemorrhagic telangiectasia 2(HHT2).

The TGFβ (transforming growth factor β) superfamily proteins are pleiotropic cytokines that regulate a diverse range of cellular processes, including proliferation, differentiation, migration, adhesion and death.  The TGFβ superfamily elicits 4 signaling pathways: TGFβ, Bone Morphogenic Protein (BMP), Activin, and Nodal.  Each pathway signals through a heteromeric receptor complex composed of at least two type-I and two type-II transmembrane receptors containing cytoplasmic serine/threonine kinase domains. These receptors are distinguished by the presence of a glycine/serine-rich juxta-membrane domain found only in the type I receptors. Either type receptor may initially bind ligand, followed by the recruitment of an alternate-type receptor counterpart to form a signal-activating complex. The type I receptors are also referred to as ALKs (Activin receptor-like kinases). ACVRL1 (Activin A receptor type II-like 1), also known as ALK1, HHT2, SKR3, TSR-I, and ACVRLK1, is an endothelial-specific type I receptor in the TGFβ receptor (TGFBR) family. ACVRL1 is expressed highly in endothelial cells and other highly vascularized tissues. ACVRL1 acts as a type I receptor for TGFβ1, TGFβ3, BMP9 and BMP10 and an important regulator of normal blood vessel development or repair. It may bind Activin as well.  The expression pattern of ALK­1 parallels that of endoglin. Mutations in ACVRL1 as well as in endoglin are associated with hemorrhagic telangiectasia 2(HHT2).

 

Product Details

 

Gene Symbol: ACVRL1; ALK1; ALK-1; HHT; HHT2; ORW2; SKR3; ALK-1; TSR-I; ACVRLK1

 

NCBI Gene ID: 94

 

Uniprot Entry: P37023

 

Construct Details: The recombinant humanACVRL1-Fc fusion protein is expressed as a 325 amino acid protein consisting of Asp22 - Gln118 region of ACVRL1 (UniProt accession #P37023) and a C-terminal Fc fusion from human IgG1, which exists as a dimer under non-reducing condition (see the gel image above, labeled as "DTT: -").

 

Source: Human cells stably expressing human ACVRL1-Fc and growing in chemical-defined media with no animal components or antibiotics

 

Deduced Amino Acid Sequence:

DPVKPSRGPLVTCTCESPHCKGPTCRGAWCTVVLVREEGRHPQEHRGCG

NLHRELCRGRPTEFVNHYCCDSHLCNHNVSLVLEATQPPSEQPGTDGQS

TGTHTCPPCPAPELLGGPSVFLFPPKPKDTLMISRTPEVTCVVVDVSHE

DPEVKFNWYVDGVEVHNAKTKPREEQYNSTYRVVSVLTVLHQDWLNGKE

YKCKVSNKALPAPIEKTISKAKGQPREPQVYTLPPSREEMTKNQVSLTC

LVKGFYPSDIAVEWESNGQPENNYKTTPPVLDSDGSFFLYSKLTVDKSR

WQQGNVFSCSVMHEALHNHYTQKSLSLSPGK

 

M.W.: Calculated molecular mass (kDa): 36.3; Estimated by SDS-PAGE under reducing condition (kDa): ~50

 

Calculated PI: 6.97

 

Calculated extinction coefficients (M-1 cm-1, at 280nm):  43400

 

Purity: >95% judged by SDS-PAGE under reducing condition (see the gel image above, labeled as "DTT: +")

 

Formulation: Supplied at 0.5 mg/ml in sterile PBSpH7.4 (carrier & preservative free). The purified recombinant protein was labeled with Biotin (3-5 Biotin per molecule) using the standard procedure.

 

Endotoxin Level: <0.1 EU per 1 μg of purified recombinant protein determined by the LAL method

 

Biological Activity: Immobilized ACVRL1/ALK1 protein binds human BMP9 in a functional ELISA.  Inhibits BMP9-induced alkaline phosphatase production by chondrogenic cells. 

 

 

 

 

  

FOR RESEARCH USE ONLY. NOT FOR DIAGNOSTIC OR THERAPEUTIC USE IN HUMAN.

Restriction: This product is not transferable or re-sellable.  Customer obtain no right to transfer, assign, or sublicense its use rights, or to transfer, resell, package, or otherwise distribute the product, or to use the product for the benefit of any third party or for any commercial purpose.  Customer may only use the product in compliance with applicable local, state and federal laws, regulations and rules.  Customer may not directly or indirectly use the product or allow the transfer, transmission, export or re-export of all or any part of the product in violation of any export control law or regulation of the united states or any other relevant jurisdiction.  Your use of this product constitutes acceptance of the terms of this limited use agreement.  Please refer to our “terms & conditions” for details.  If you are not willing to accept the limitation of this agreement, G&P Biosciences will accept return of the product for a full/partial refund.

 

Storage

 

The product is shipped at 4°C. Upon receipt, centrifuge the product briefly before opening the vial. It is recommended to store small aliquots at the temperature below –20°C for long-term storage and the product is stable for 3 months. The undiluted protein can be stored at 4°C for no more than 2 weeks.

 

 

Use a manual defrost freezer and avoid repeated freeze-thaw cycles.

 

 

 

FOR RESEARCH USE ONLY. NOT FOR DIAGNOSTIC OR THERAPEUTIC USE IN HUMAN.

 

References

 

1. Oncogene 8:2879 (1993).  

2. Science 264:101 (1994).  

3. J. Biol. Chem. 274:9984 (1999). 

4. Biochemistry 51:6328 (2012).

5. J. Biol. Chem. 287:27313 (2012).

6. Nat. Genet. 13:189 (1996).

 

 

Documentation

 

Product datasheet (pdf) can be downloaded here: FCL0124B-PDS.pdf

 

 

 

Additional supporting documents, including COA and MSDS are available upon request.

 

 

 

FOR RESEARCH USE ONLY. NOT FOR DIAGNOSTIC OR THERAPEUTIC USE IN HUMAN.

Restriction: This product is not transferable or re-sellable.  Customer obtain no right to transfer, assign, or sublicense its use rights, or to transfer, resell, package, or otherwise distribute the product, or to use the product for the benefit of any third party or for any commercial purpose.  Customer may only use the product in compliance with applicable local, state and federal laws, regulations and rules.  Customer may not directly or indirectly use the product or allow the transfer, transmission, export or re-export of all or any part of the product in violation of any export control law or regulation of the united states or any other relevant jurisdiction.  Your use of this product constitutes acceptance of the terms of this limited use agreement.  Please refer to our “terms & conditions” for details.  If you are not willing to accept the limitation of this agreement, G&P Biosciences will accept return of the product for a full/partial refund.