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). ACVR1 (Activin receptor type-1), also known as ACVR1A, ALK2, FOP, SKR1, TSRI, ACVRLK2, and ActRI, is a type I receptor in the TGFβ receptor family.  ACVR1 was identified as a receptor for both Activin and BMP.  It is expressed in normal parenchymal cells, endothelial cells, fibroblasts and tumor-derived epithelial cells.  ACVR1 plays a critical role in gastrulation, skeletal system development, and cardiac morphogenesis. It may be required for left-right pattern formation during embryogenesis.  Overexpression of ACVR1 causes a mitotic arrest. ACVR1 can phosphorylate the co-receptor Endoglin and mediate the inhibitory effect of Endoglin on prostate cancer cell motility.  Defects in ACVR1 are associated with fibrodysplasia ossificans progressiva (FOP).

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). ACVR1 (Activin receptor type-1), also known as ACVR1A, ALK2, FOP, SKR1, TSRI, ACVRLK2, and ActRI, is a type I receptor in the TGFβ receptor family.  ACVR1 was identified as a receptor for both Activin and BMP.  It is expressed in normal parenchymal cells, endothelial cells, fibroblasts and tumor-derived epithelial cells.  ACVR1 plays a critical role in gastrulation, skeletal system development, and cardiac morphogenesis. It may be required for left-right pattern formation during embryogenesis.  Overexpression of ACVR1 causes a mitotic arrest. ACVR1 can phosphorylate the co-receptor Endoglin and mediate the inhibitory effect of Endoglin on prostate cancer cell motility.  Defects in ACVR1 are associated with fibrodysplasia ossificans progressiva (FOP).

 

Product Details

 

Gene Symbol: ACVR1; ALK2; FOP; ALK-2; SKR1; TSRI; TSR-I; ACTRI; ActR-I;  ACVR1A; ACVRLK2; ACVRIA; ACVR-IA

 

NCBI Gene ID: 90

 

Uniprot Entry: Q04771

 

Construct Details: The recombinant human ACVR1-Fc fusion protein is expressed as a 331amino acid protein consisting of Met21 - Glu123 region of ACVR1 (UniProt accession #Q04771) 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 ACVR1-Fc and growing in chemical-defined media with no animal components or antibiotics

 

Deduced Amino Acid Sequence:

MEDEKPKVNPKLYMCVCEGLSCGNEDHCEGQQCFSSLSINDGFHVYQKGC

FQVYEQGKMTCKTPPSPGQAVECCQGDWCNRNITAQLPTKGKSFPGTQNF

HLESTGTHTCPPCPAPELLGGPSVFLFPPKPKDTLMISRTPEVTCVVVDV

SHEDPEVKFNWYVDGVEVHNAKTKPREEQYNSTYRVVSVLTVLHQDWLNG

KEYKCKVSNKALPAPIEKTISKAKGQPREPQVYTLPPSREEMTKNQVSLT

CLVKGFYPSDIAVEWESNGQPENNYKTTPPVLDSDGSFFLYSKLTVDKSR

WQQGNVFSCSVMHEALHNHYTQKSLSLSPGK

 

 

M.W.: Calculated molecular mass (kDa): 37.1; Estimated by SDS-PAGE under reducing condition (kDa): ~45

 

Calculated PI: 6.37

 

Calculated extinction coefficients (M-1 cm-1, at 280nm):  46380

 

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 ACVR1 protein binds human BMP6 in a functional ELISA.  Blocks BMP6-mediated signaling activity.

 

 

 

 

  

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. Cell 75:671 (1993). 

2. J. Biol. Chem. 268:12719 (1993).

3. Oncogene 8:2879 (1993).

4. Genes Dev. 18:1838 (2004). 

5. Nat. Genet. 38:525 (2006).

 

 

Documentation

 

Product datasheet (pdf) can be downloaded here: FCL0125B-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.