克隆類型 : |
Rabbit Polyclonal IgG |
免 疫 原 : |
Peptide derived from Hu BMPR1B. |
純化方式 : |
Immunogen affinity purified |
緩 沖 液 : |
PBS, pH 7.4(1%BSA and 0.1% Sodium azide) |
產(chǎn)品描述 : |
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). |
適用物種 : |
Hu, Mo, Rat, Dog, Cow, Sheep, |
應(yīng)用說明 : |
WB: 1:200-1:1000 E: 1:500-1:1000 IHC: 1:200-1:1000 IF: 1:100-1:500 |
別 名 : |
BMPR-IB; Activin receptor like kinase 6; Acvrlk6; ALK6; alk6tr; BMP type-1B receptor; Bmpr1b; BMPRIB; BMR1B_Hu; Bone morphogenetic protein receptor type 1B; BR1b; CDw293; CDw293 antigen; CFK43a; Serine/threonine receptor kinase; zALK6. |
保存條件 : |
Store at -20°C for one year. Avoid repeated freeze-thaw cycles. At 4°C for 2-3 weeks after reconstituted. |
標(biāo) 記 物 : |
PLLABS公司可供應(yīng)生物素、辣根過氧化物酶、熒光素標(biāo)記抗體 |
產(chǎn)品說明 : |
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