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Rabbit Anti-PITX3/BF594 Conjugated antibody (bs-2364R-BF594)
訂購熱線:400-901-9800
訂購郵箱:sales@bioss.com.cn
訂購QQ:  400-901-9800
技術支持:techsupport@bioss.com.cn
說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產品編號 bs-2364R-BF594
英文名稱 Rabbit Anti-PITX3/BF594 Conjugated antibody
中文名稱 BF594標記的炎癥因子3/穿透素抗體
別    名 Homeobox protein PITX 3; Homeobox protein PITX3; MGC12766; Paired like homeodomain transcription factor 3; Paired-like homeodomain transcription factor 3; Pituitary homeobox 3; PITX 3; Pitx3; PITX3_HUMAN; PTX 3; PTX3.  
規格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領域 細胞生物  免疫學  染色質和核信號  神經生物學  表觀遺傳學  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 Mouse, Rat, Cow,  (predicted: Human, Chicken, Dog, Pig, Horse, Rabbit, )
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 32kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human PITX3
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:
The transcription factor PITX3 is expressed selectively in the midbrain and regulates the differentiation and survival of dopaminergic neurons. Lack of this factor results in a degeneration similar to that seen in Parkinson's disease. PITX3 is also important in eye developement; mutations of the PITX3 gene have been associated with a familial form of cataracts.

Function:
Transcriptional regulator which is important for the differentiation and maintenance of meso-diencephalic dopaminergic (mdDA) neurons during development. In addition to its importance during development, it also has roles in the long-term survival and maintenance of the mdDA neurons. Activates NR4A2/NURR1-mediated transcription of genes such as SLC6A3, SLC18A2, TH and DRD2 which are essential for development of mdDA neurons. Acts by decreasing the interaction of NR4A2/NURR1 with the corepressor NCOR2/SMRT which acts through histone deacetylases (HDACs) to keep promoters of NR4A2/NURR1 target genes in a repressed deacetylated state. Essential for the normal lens development and differentiation. Plays a critical role in the maintenance of mitotic activity of lens epithelial cells, fiber cell differentiation and in the control of the temporal and spatial activation of fiber cell-specific crystallins. Positively regulates FOXE3 expression and negatively regulates PROX1 in the anterior lens epithelium, preventing activation of CDKN1B/P27Kip1 and CDKN1C/P57Kip2 and thus maintains lens epithelial cells in cell cycle.

Subunit:
Interacts with SFPQ.

Subcellular Location:
Nucleus.

Tissue Specificity:
Highly expressed in developing eye lens.

DISEASE:
Defects in PITX3 are a cause of cataract autosomal dominant (ADC) [MIM:604219]. Cataract is an opacification of the crystalline lens of the eye that frequently results in visual impairment or blindness. Opacities vary in morphology, are often confined to a portion of the lens, and may be static or progressive. In general, the more posteriorly located and dense an opacity, the greater the impact on visual function. Cataract is the most common treatable cause of visual disability in childhood.
Defects in PITX3 are a cause of anterior segment mesenchymal dysgenesis (ASMD) [MIM:107250]; also known as anterior segment ocular dysgenesis (ASOD). ASMD consists of a range of developmental defects in structures at the front of the eye, resulting from abnormal migration or differentiation of the neural crest derived mesenchymal cells that give rise to the cornea, iris, and other components of the anterior chamber during eye development. Mature anterior segment anomalies are associated with an increased risk of glaucoma and corneal opacity. Conditions falling within the phenotypic spectrum include aniridia, posterior embryotoxon, Axenfeld anomaly, Reiger anomaly/syndrome, Peters anomaly, and iridogoniodysgenesis.
Defects in PITX3 are the cause of cataract posterior polar type 4 (CTPP4) [MIM:610623]. A subcapsular opacity, usually disk-shaped, located at the back of the lens. It can have a marked effect on visual acuity. Some patients affected by cataract posterior polar type 4 can present a severe phenotype including microphthalmia and neurological dysfunction.

Similarity:
Belongs to the paired homeobox family. Bicoid subfamily.
Contains 1 homeobox DNA-binding domain.

Database links:

Entrez Gene: 5309 Human

Entrez Gene: 18742 Mouse

Entrez Gene: 29609 Rat

Omim: 602669 Human

SwissProt: O75364 Human

SwissProt: Q5VZL2 Human

SwissProt: O35160 Mouse

SwissProt: P81062 Rat

Unigene: 137568 Human

Unigene: 6255 Mouse

Unigene: 22092 Rat



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

PITX3是一種急性期反應蛋白,也是一種炎癥標志物,在炎癥級聯反應中均起著重要作用,并參與了機械牽張刺激引起的炎癥反應過程。
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