BMPR1B Polyclonal Antibody, Cy7 Conjugated

Catalogue Number: BS-6639R-CY7-BSS

Manufacturer:	Bioss Inc
Type:	Polyclonal Primary Antibody - Conjugated
Shipping Condition:	Blue Ice
Unit(s):	100 ul
Host name:	Rabbit
Clone:
Isotype:	IgG
Immunogen:	KLH conjugated synthetic peptide derived from human BMPR1B
Application:	WB

Overview
Datasheets

Description

Description: 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.

Additional Text

Gene Name

BMPR1B

Gene ID

658

Purification

Protein A purified

Concentration

1 ug/ul

Antibody Clonality

Polyclonal

Modification

Unmodified

Specificity

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.

Storage Note

Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.

Excitation

743nm/767nm

BS-6639R-CY7 Datasheet

Price

£390.00

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