骨形態發生蛋白受體1B抗體

產品貨號：

BN41945R
中文名稱：

骨形態發生蛋白受體1B抗體
英文名稱：

Rabbit anti-BMPR1B Polyclonal antibody
品牌：

Biorigin

貨號

產品規格

售價

備注
BN41945R-50ul

50ul

￥1486.00

交叉反應：nan(predicted:Human,Mouse,Rat,Dog,Cow,Rabbit,Sheep) 推薦應用：WB,ELISA
BN41945R-100ul

100ul

￥2360.00

交叉反應：nan(predicted:Human,Mouse,Rat,Dog,Cow,Rabbit,Sheep) 推薦應用：WB,ELISA
BN41945R-200ul

200ul

￥3490.00

交叉反應：nan(predicted:Human,Mouse,Rat,Dog,Cow,Rabbit,Sheep) 推薦應用：WB,ELISA

產品描述

英文名稱	BMPR1B
中文名稱	骨形態發生蛋白受體1B抗體
別名	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.
研究領域	細胞生物信號轉導干細胞轉錄調節因子激酶和磷酸酶細胞表面分子細胞外基質表觀遺傳學
抗體來源	Rabbit
克隆類型	Polyclonal
交叉反應	(predicted: Human, Mouse, Rat, Dog, Cow, Rabbit, Sheep, .)
產品應用	WB=1:500-2000 ELISA=1:5000-10000 not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
分子量	56kDa
細胞定位	細胞膜
性狀	Liquid
濃度	1mg/ml
免疫原	KLH conjugated synthetic peptide derived from human BMPR1B:61-160/502 <Extracellular>
亞型	IgG
純化方法	affinity purified by Protein A
儲存液	0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存條件	Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
PubMed	PubMed
產品介紹	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. 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. Subcellular Location: Membrane; Single-pass type I membrane protein. 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. Similarity: Belongs to the protein kinase superfamily. TKL Ser/Thr protein kinase family. TGFB receptor subfamily. Contains 1 GS domain. Contains 1 protein kinase domain. SWISS: O00238 Gene ID: 658 Database links: Entrez Gene: 658 Human Entrez Gene: 12167 Mouse Omim: 603248 Human SwissProt: O00238 Human SwissProt: P36898 Mouse Unigene: 598475 Human Unigene: 39089 Mouse Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications