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Rabbit Anti-GATA6/BF350 Conjugated antibody (bs-1787R-BF350)
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產品編號 bs-1787R-BF350
英文名稱1 Rabbit Anti-GATA6/BF350 Conjugated antibody
中文名稱 BF350標記的GATA結合蛋白6抗體
別    名 Gata binding factor 6; Gata binding protein 6; GATA-binding factor 6; Gata6; GATA6_HUMAN; Transcription factor Gata 6; Transcription factor GATA-6.  
規格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領域 腫瘤  心血管  細胞生物  免疫學  發育生物學  染色質和核信號  干細胞  轉錄調節因子  結合蛋白  表觀遺傳學  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 Human,  (predicted: Mouse, Rat, Chicken, Dog, Pig, Cow, Rabbit, Sheep, )
產品應用 ICC=1:50-200 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 60kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human GATA-6
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:
This gene is a member of a small family of zinc finger transcription factors that play an important role in the regulation of cellular differentiation and organogenesis during vertebrate development. This gene is expressed during early embryogenesis and localizes to endo- and mesodermally derived cells during later embryogenesis and thereby plays an important role in gut, lung, and heart development. Mutations in this gene are associated with several congenital defects. [provided by RefSeq, Mar 2012].

Function:
Transcriptional activator that regulates SEMA3C and PLXNA2. Thought to be important for regulating terminal differentiation and/or proliferation.

Subunit:
Interacts with LMCD1 (By similarity).

Subcellular Location:
Nucleus.

Tissue Specificity:
Expressed in heart, gut and gut-derived tissues.

DISEASE:
Defects in GATA6 are a cause of conotruncal heart malformations (CTHM) [MIM:217095]. A group of congenital heart defects involving the outflow tracts. Examples include truncus arteriosus communis, double-outlet right ventricle and transposition of great arteries. Truncus arteriosus communis is characterized by a single outflow tract instead of a separate aorta and pulmonary artery. In transposition of the great arteries, the aorta arises from the right ventricle and the pulmonary artery from the left ventricle. In double outlet of the right ventricle, both the pulmonary artery and aorta arise from the right ventricle. Note=GATA6 mutations have been found in patients with non-syndromic persistent truncus arteriosus (PubMed:19666519).
Defects in GATA6 are the cause of atrial septal defect type 9 (ASD9) [MIM:614475]. A congenital heart malformation characterized by incomplete closure of the wall between the atria resulting in blood flow from the left to the right atria. Some patients manifest tricuspid valve disease, pulmonary valve disease, and pulmonary artery hypertension.
Defects in GATA6 are a cause of tetralogy of Fallot (TOF) [MIM:187500]. A congenital heart anomaly which consists of pulmonary stenosis, ventricular septal defect, dextroposition of the aorta (aorta is on the right side instead of the left) and hypertrophy of the right ventricle. In this condition, blood from both ventricles (oxygen-rich and oxygen-poor) is pumped into the body often causing cyanosis.
Defects in GATA6 are the cause of atrioventricular septal defect type 5 (AVSD5) [MIM:614474]. A congenital heart malformation characterized by a common atrioventricular junction coexisting with deficient atrioventricular septation. The complete form involves underdevelopment of the lower part of the atrial septum and the upper part of the ventricular septum; the valve itself is also shared. A less severe form, known as ostium primum atrial septal defect, is characterized by separate atrioventricular valvar orifices despite a common junction.
Defects in GATA6 are a cause of pancreatic agenesis and congenital heart defects (PACHD) [MIM:600001]. An autosomal dominant disease characterized by pancreatic severe hypoplasia or agenesis, diabetes mellitus, and congenital heart abonormalities including ventricular septal defect, patent ductus arteriosus, pulmonary artery stenosis, truncus arteriosus and tetralogy of Fallot.

Similarity:
Contains 2 GATA-type zinc fingers.

Database links:

Entrez Gene: 2627 Human

Entrez Gene: 14465 Mouse

Entrez Gene: 29300 Rat

Omim: 601656 Human

SwissProt: Q92908 Human

SwissProt: Q61169 Mouse

SwissProt: P46153 Rat

Unigene: 514746 Human

Unigene: 329287 Mouse

Unigene: 8701 Rat



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

GATA6又稱轉錄調節因子GATA6,可能抑制Wnt路徑,方式是通過直接調控Wnt路徑中另一種名為Fzd2的蛋白的表達。而Wnt路徑是干細胞生物學中一個主要的路徑。GATA6負調控Wnt路徑并且其已被證明在胚胎干細胞復制和分化中也起重要作用。
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