BMPR1B Polyclonal Antibody

• Background:

  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) . 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.

• Synonyms:

  BMPR-IB; 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.

• Gene ID:

  658

• Cellular Locus:

  Cell membrane

• Host:

  Rabbit

• Cross Reactivity:

  Human

• Immunogen:

  61-160/502

• Target:

  BMPR1B

• Clonality:

  Polyclonal

• Isotype:

  IgG

• Conjugation:

  Unconjugated

• Source:

  KLH conjugated synthetic peptide derived from human BMPR1B

• Applications:

  WB, ELISA

• Purification:

  Purified by Protein A.

• Concentration:

  1µg/µl

• Dilution:

  WB (1:300-5000), ELISA (1:500-1000)

• Buffer:

  0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.

• Modification:

  Unmodified

• Storage Conditions:

  Shipped at 4C. Store at -20C for one year. Avoid repeated freeze/thaw cycles.

• Gene ID URL:

  658

• Predicted Cross Reactivity:

  Mouse, Rat, Dog, Cow, Sheep, Rabbit