?骨形态发生蛋白受体1B抗体Quantity size:
0.2ml
Concentration:
1mg/ml Buffer = 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
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,骨形态发生蛋白受体1B抗体 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.骨形态发生蛋白受体1B抗体 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.骨形态发生蛋白受体1B抗体 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.
Also known as:
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.
Specificity:
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Rabbit Polyclonal IgG, affinity purified by Protein A.
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Reacts with: Human, Mouse, Rat, Dog, Cow, Rabbit, Sheep, ..
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Immunogen: KLH conjugated synthetic peptide derived from human BMPR1B.
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骨形态发生蛋白受体1B抗体Predicted Molecular Weight: 54kDa.
Storage:
Shipped at 4℃, Store at -20℃ (Avoid repeated freeze/thaw cycles).
Application:
WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:100-500
Not yet tested in other applications.
Optimal working dilutions must be determined by the end user.
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kl099Ga01基质金属蛋白酶13(MMP13)多克隆抗体Polyclonal Antibody to Matrix Metalloproteinase 13 (MMP13)
kl099Hu01基质金属蛋白酶13(MMP13)多克隆抗体Polyclonal Antibody to Matrix Metalloproteinase 13 (MMP13)
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kl101Bo01基质金属蛋白酶3(MMP3)多克隆抗体Polyclonal Antibody to Matrix Metalloproteinase 3 (MMP3)
kl101Ca01基质金属蛋白酶3(MMP3)多克隆抗体Polyclonal Antibody to Matrix Metalloproteinase 3 (MMP3)
kl101Eq01基质金属蛋白酶3(MMP3)多克隆抗体Polyclonal Antibody to Matrix Metalloproteinase 3 (MMP3)
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企业名称
上海康朗生物科技有限公司
企业信息已认证
企业类型
信用代码
310112001510759
成立日期
2015-05-29
注册资本
100
经营范围
从事生物科技技术领域内技术开发、技术咨询、技术服务、技术转让,实验分析仪器、化工产品及原料(除危险化学品、监控化学品、烟花爆竹、民用爆竹物品、易制毒化学品)、仪器仪表、环保设备、酒店用品、皮革制品、床上用品、电子产品的销售。【依法须经批准的项目,经相关部门批准后方可开展经营活动】
上海康朗生物科技有限公司
公司地址
上海市闵行区闵北路88弄1-30号第22栋BV145室
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