BMPR1B anticorps (AA 61-160) (HRP)

N° du produit ABIN719255

Détail du produit anti-BMPR1B anticorps (HRP)

Antigène: BMPR1B (Bone Morphogenetic Protein Receptor, Type IB (BMPR1B))

Épitope: AA 61-160

Reactivité: Humain

Hôte: Lapin

Clonalité: Polyclonal

Conjugué: Cet anticorp BMPR1B est conjugé à/à la HRP

Application: Western Blotting (WB), ELISA

Homologie: Human,Mouse,Rat,Dog,Cow,Sheep,Rabbit

Purification: Purified by Protein A.

Immunogène: KLH conjugated synthetic peptide derived from human BMPR1B

Isotype: IgG

Information d'application

Indications d'application: WB 1:300-5000

Restrictions: For Research Use only

Stockage

Format: Liquid

Concentration: 1 μg/μL

Buffer: Aqueous buffered solution containing 0.01M TBS ( pH 7.4) with 1 % BSA, 0.03 % Proclin300 and 50 % Glycerol.

Agent conservateur: ProClin

Précaution d'utilisation: This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.

Conseil sur la manipulation: Do NOT add Sodium Azide! Use of Sodium Azide will inhibit enzyme activity of horseradish peroxidase.

Stock: -20 °C

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

Date de péremption: 12 months

Détails sur BMPR1B

Antigène: BMPR1B (Bone Morphogenetic Protein Receptor, Type IB (BMPR1B))

Autre désignation: BMPR1B (BMPR1B Produits)

Synonymes: anticorps ALK6, anticorps BMPR-IB, anticorps FecB, anticorps alk-6, anticorps alk6, anticorps cdw293, anticorps alk6b, anticorps zgc:172219, anticorps BMPR1B, anticorps AI385617, anticorps ALK-6, anticorps AV355320, anticorps Acvrlk6, anticorps Alk6, anticorps BMPR-1B, anticorps CFK-43a, anticorps SKR6, anticorps BR1b, anticorps alk6tr, anticorps bmpr1b, anticorps zALK-6, anticorps zgc:92220, anticorps CDw293, anticorps BMP15, anticorps BMPRIB, anticorps RPK-1, anticorps bone morphogenetic protein receptor type 1B, anticorps bone morphogenetic protein receptor, type IBb, anticorps bone morphogenetic protein receptor, type 1B, anticorps bone morphogenetic protein receptor, type IBa, anticorps BMPR1B, anticorps bmpr1b, anticorps bmpr1bb, anticorps Bmpr1b, anticorps bmpr1ba

Sujet:

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.

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.

ID gène: 658