Recombinant Human BMPR1B protein (ab152236)

Overview

Description

  • Nature
    Recombinant
  • Source
    Wheat germ
  • Amino Acid Sequence
    • Accession
    • Species
      Human
    • Sequence
      KKEDGESTAPTPRPKVLRCKCHHHCPEDSVNNICSTDGYCFTMIEEDDSG LPVVTSGCLGLEGSDFQCRDTPIPHQRRSIECCTERNECN
    • Molecular weight
      36 kDa including tags
    • Amino acids
      14 to 103

Specifications

Our Abpromise guarantee covers the use of ab152236 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

  • Applications

    Western blot

    SDS-PAGE

    ELISA

  • Form
    Liquid
  • Additional notes
    Protein concentration is above or equal to 0.05 mg/ml.
  • Concentration information loading...

Preparation and Storage

  • Stability and Storage

    Shipped on dry ice. Upon delivery aliquot and store at -80ºC. Avoid freeze / thaw cycles.

    pH: 8.00
    Constituents: 0.31% Glutathione, 0.79% Tris HCl

General Info

  • Alternative names
    • Activin receptor like kinase 6
    • Acvrlk6
    • ALK 6
    • ALK6
    • alk6tr
    • BMP type-1B receptor
    • BMPR IB
    • BMPR-1B
    • Bmpr1b
    • BMPRIB
    • BMR1B_HUMAN
    • Bone morphogenetic protein receptor type 1B
    • Bone morphogenetic protein receptor type IB
    • Bone morphogenetic protein receptor type-1B
    • BR 1b
    • BR1b
    • CDw 293
    • CDw293
    • CDw293 antigen
    • CFK 43a
    • CFK43a
    • Serine/threonine receptor kinase
    • zALK 6
    • zALK6
    see all
  • 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.
  • Involvement in disease
    Defects in BMPR1B are the cause of acromesomelic chondrodysplasia with genital anomalies (AMDGA) [MIM:609441]. 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).
    Defects in BMPR1B are a cause of brachydactyly type A2 (BDA2) [MIM:112600]. Brachydactylies (BDs) are a group of inherited malformations characterized by shortening of the digits due to abnormal development of the phalanges and/or the metacarpals. They have been classified on an anatomic and genetic basis into five groups, A to E, including three subgroups (A1 to A3) that usually manifest as autosomal dominant traits. BDA2 was described first in a large Norwegian kindred. BDA2 is caused by mutations in BMPR1B gene and studies demonstrate that these mutations function as dominant negatives in vitro and in vivo.
  • 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.
  • Cellular localization
    Membrane.
  • Information by UniProt

Images

  • 12.5% SDS-PAGE analysis of ab152236 stained with Coomassie Blue.

References

ab152236 has not yet been referenced specifically in any publications.

Customer reviews and Q&As

There are currently no Customer reviews or Questions for ab152236.
Please use the links above to contact us or submit feedback about this product.

Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"

Sign up