BMPR1B Antikörper (AA 61-160)

Produktnummer ABIN719246

Der Kaninchen Polyklonal anti-BMPR1B Antikörper wird verwendet zum Nachweis von BMPR1B in Proben von Human. Er wurde validiert für WB und ELISA.

Kurzübersicht für BMPR1B Antikörper (AA 61-160) (ABIN719246)

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

Reaktivität: Human

Wirt: Kaninchen

Klonalität: Polyklonal

Konjugat: Dieser BMPR1B Antikörper ist unkonjugiert

Applikation: Western Blotting (WB), ELISA

Produktdetails anti-BMPR1B Antikörper

Bindungsspezifität: AA 61-160

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

Aufreinigung: Purified by Protein A.

Immunogen: KLH conjugated synthetic peptide derived from human BMPR1B

Isotyp: IgG

Anwendungsinformationen

Applikationshinweise: WB 1:300-5000
ELISA 1:500-1000

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

Format: Liquid

Konzentration: 1 μg/μL

Buffer: 0.01M TBS( pH 7.4) with 1 % BSA, 0.02 % Proclin300 and 50 % Glycerol.

Konservierungsmittel: ProClin

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

Lagerung: 4 °C,-20 °C

Informationen zur Lagerung: Shipped at 4°C. Store at -20°C for one year. Avoid repeated freeze/thaw cycles.

Haltbarkeit: 12 months

Details zu BMPR1B

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

Andere Bezeichnung: BMPR1B

Hintergrund:

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.

Gen-ID: 658