巴尔得-别德尔综合征相关蛋白9抗体

?巴尔得-别德尔综合征相关蛋白9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:

BBS9 is an 887 amino acid protein that localizes to both the cytoplasm and the centrosome and exists as six alternatively spliced isoforms. Expressed in a wide variety of tissues, including liver, lung, heart,巴尔得-别德尔综合征相关蛋白9抗体 brain and skeletal muscle, BBS9 functions as a component of the multi-protein BBSome complex which is required for ciliogenesis and is regulated by GDP/GTP exchange factors. Defects in the gene encoding BBS9 are associated with the pathogenesis of Bardet-Biedl syndrome type 9 (BBS9), an autosomal recessive disorder that is characterized by severe pigmentary retinopathy,巴尔得-别德尔综合征相关蛋白9抗体 early onset obesity, polydactyly, hypogenitalism, renal malformation and mental retardation. Additionally, chromosomal aberrations involving the BBS9 gene may play a role in the formation of Wilms tumor 5 (WT5).

Also known as:

巴尔得-别德尔综合征相关蛋白9抗体B1 antibody; Bardet Biedl syndrome 9; Bardet-Biedl syndrome 9 protein; bbs9; C18 antibody D1 antibody MGC118917; 1 gene protein; Protein PTHB1; PTH-responsive osteosarcoma B1 protein; PTHB1; PTHB1_HUMAN.

Specificity:

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Rabbit Polyclonal IgG, affinity purified by Protein A.

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Reacts with: Human, Mouse, Rat, Dog, Pig, Horse, Rabbit, Sheep, .

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Immunogen: KLH conjugated synthetic peptide derived from human BBS9.

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巴尔得-别德尔综合征相关蛋白9抗体Predicted Molecular Weight: 99kDa.

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  ICC=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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