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Rabbit Anti-PAX2/RBITC Conjugated antibody (bs-1187R-RBITC)
~~~促销,代码KXJ230206-6~~~
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说 明 书: 100ul  
100ul/2980.00元
大包装/询价
产品编号 bs-1187R-RBITC
英文名称 Anti-PAX2/RBITC
中文名称 罗丹明(RBITC)标记的配对盒基因2抗体
别    名 PAX2 Paired box gene 2; Paired box gene 2; paired box homeotic gene 2; paired box protein 2; Paired box protein Pax 2; Paired box protein Pax-2; Paired box protein Pax2; Pax 2.  
规格价格 100ul/2980元 购买        大包装/询价
说 明 书 100ul  
研究领域 肿瘤  转录调节因子  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Mouse, Rat,  (predicted: Human, Chicken, Dog, Pig, Cow, Horse, Rabbit, Sheep, )
产品应用 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 46kDa
性    状 Lyophilized or Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human PAX2
亚    型 IgG
纯化方法 affinity purified by Protein A
储 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件 Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
产品介绍 background:
PAX2 encodes paired box gene 2, one of many human homologues of the Drosophila melanogaster gene prd. The central feature of this transcription factor gene family is the conserved DNA-binding paired box domain. PAX2 is believed to be a target of transcriptional supression by the tumor supressor gene WT1. Mutations within PAX2 have been shown to result in optic nerve colobomas and renal hypoplasia. Alternative splicing of this gene results in multiple transcript variants. [provided by RefSeq, Jul 2008]

Function:
Probable transcription factor that may have a role in kidney cell differentiation. Has a critical role in the development of the urogenital tract, the eyes, and the CNS.

Subunit:
Interacts with ELGN3; the interaction targets PAX2 for destruction.

Subcellular Location:
Nuclear.

Tissue Specificity:
Expressed in primitive cells of the kidney, ureter, eye, ear and central nervous system.

DISEASE:
Renal-coloboma syndrome (RCS) [MIM:120330]: An autosomal dominant disorder characterized by both ocular and renal anomalies, but may also include vesicoureteral reflux, high frequency hearing loss, central nervous system anomalies, and/or genital anomalies. Eye anomalies in this disorder consist of a wide and sometimes excavated dysplastic optic disk with the emergence of the retinal vessels from the periphery of the disk, designated optic nerve coloboma or 'morning glory' anomaly. Associated findings may include a small corneal diameter, retinal coloboma, sclera staphyloma, optic nerve cyst, microphthalmia, and pigmentary macular dysplasia. The kidneys are small and abnormally formed (renal hypodysplasia), and have fewer than the normal number of glomeruli, which are enlarged (oligomeganephronia). These ocular and renal anomalies result in decreased visual acuity and retinal detachment, as well as hypertension, proteinuria, and renal insufficiency that frequently progresses to end-stage renal disease. Note=The disease is caused by mutations affecting the gene represented in this entry.
Note=Defects in PAX2 can be responsible for isolated renal hypodysplasia and oligomeganephronia (OMN). This is a rare congenital and usually sporadic anomaly characterized by bilateral renal hypoplasia, with a reduced number of enlarged nephrons and without urinary tract abnormalities.

Similarity:
Contains 1 paired domain.

Database links:

Entrez Gene: 5076 Human

Entrez Gene: 18504 Mouse

Omim: 167409 Human

SwissProt: Q02962 Human

SwissProt: P32114 Mouse

Unigene: 155644 Human

Unigene: 192158 Mouse



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

同源结构域蛋白(Homeodomain Proteins)
PAX2 可结合DNA的位点,属转录抑制因子。PAX2基因在介导雌激素和三苯氧胺刺激的子宫内膜细胞的增殖和癌变过程中起着关键作用。
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