巴尔得-别德尔综合征相关蛋白2抗体

?巴尔得-别德尔综合征相关蛋白2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:

Bardet-Biedl syndrome (BBS) is a pleiotropic genetic disorder characterized by obesity, photoreceptor degeneration, polydactyly, hypogenitalism, renal abnormalities, and developmental delay.巴尔得-别德尔综合征相关蛋白2抗体 Other associated clinical findings in BBS patients include diabetes, hypertension, and congenital heart defects. BBS is a heterogeneous disorder that maps to eight genetic loci and encodes eight proteins, BBS1-BBS8. Five BBS genes encode basal body or cilia proteins, suggesting that BBS is a ciliary dysfunction disorder. BBS2 is a 721-amino acid protein that is evolutionarily conserved and is expressed in a broad range of tissues including: brain, kidney,巴尔得-别德尔综合征相关蛋白2抗体 adrenal gland, and thyroid gland. Loss of BBS2 may be involved in defects in social interactions as well as infertility. BBS2 retinopathy involves normal retina development followed by apoptotic death of photoreceptors, the primary ciliated cells of the retina.

Also known as:

巴尔得-别德尔综合征相关蛋白2抗体Bardet Biedl syndrome 2; Bardet Biedl syndrome 2 protein; BBS; MGC20703; BBS2_HUMAN.

Specificity:

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Rabbit Polyclonal IgG, affinity purified by Protein A.

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

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Immunogen: KLH conjugated synthetic peptide derived from human BBS2.

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巴尔得-别德尔综合征相关蛋白2抗体Predicted Molecular Weight: 80kDa.

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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