骨形态发生蛋白受体1B抗体

?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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kl099Bo01基质金属蛋白酶13(MMP13)多克隆抗体Polyclonal Antibody to Matrix Metalloproteinase 13 (MMP13)

kl099Ca01基质金属蛋白酶13(MMP13)多克隆抗体Polyclonal Antibody to Matrix Metalloproteinase 13 (MMP13)

kl099Cp01基质金属蛋白酶13(MMP13)多克隆抗体Polyclonal Antibody to Matrix Metalloproteinase 13 (MMP13)

kl099Eq01基质金属蛋白酶13(MMP13)多克隆抗体Polyclonal Antibody to Matrix Metalloproteinase 13 (MMP13)

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)

kl099Mu01基质金属蛋白酶13(MMP13)多克隆抗体Polyclonal Antibody to Matrix Metalloproteinase 13 (MMP13)

kl099Po01基质金属蛋白酶13(MMP13)多克隆抗体Polyclonal Antibody to Matrix Metalloproteinase 13 (MMP13)

kl099Ra01基质金属蛋白酶13(MMP13)多克隆抗体Polyclonal Antibody to Matrix Metalloproteinase 13 (MMP13)

kl099Rb53基质金属蛋白酶13(MMP13)多克隆抗体Polyclonal Antibody to Matrix Metalloproteinase 13 (MMP13)

kl100Hu01基质金属蛋白酶2(MMP2)多克隆抗体Polyclonal Antibody to Matrix Metalloproteinase 2 (MMP2)

kl100Mu01基质金属蛋白酶2(MMP2)多克隆抗体Polyclonal Antibody to Matrix Metalloproteinase 2 (MMP2)

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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)