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Rabbit Anti-PAX2/RBITC Conjugated antibody (bs-1187R-RBITC)
訂購熱線:400-901-9800
訂購郵箱:sales@www.ssxsbs.com
訂購QQ:  400-901-9800
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產品編號 bs-1187R-RBITC
英文名稱 Rabbit Anti-PAX2/RBITC Conjugated antibody
中文名稱 羅丹明(RBITC)標記的配對盒基因2抗體
別    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.  
規格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領域 腫瘤  轉錄調節因子  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 Mouse, Rat,  (predicted: Human, Chicken, Dog, Pig, Cow, Horse, Rabbit, Sheep, )
產品應用 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    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human PAX2
亞    型 IgG
純化方法 affinity purified by Protein A
儲 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存條件 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.
產品介紹 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.

同源結構域蛋白(Homeodomain Proteins)
PAX2 可結合DNA的位點,屬轉錄抑制因子。PAX2基因在介導雌激素和三苯氧胺刺激的子宮內膜細胞的增殖和癌變過程中起著關鍵作用。
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