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Rabbit Anti-FREAC3/BF594 Conjugated antibody (bs-6642R-BF594)
訂購熱線: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-6642R-BF594
英文名稱 Rabbit Anti-FREAC3/BF594 Conjugated antibody
中文名稱 BF594標記的叉頭相關轉錄因子3/FOXC1抗體
別    名 ARA; FKH L7; FKHL 7; FKHL7; Forkhead (Drosophila) like 7; Forkhead; forkhead box C1; Forkhead box protein C1; Forkhead drosophila homolog like 7; Forkhead like 7; Forkhead related activator 3; Forkhead related protein FKHL7; Forkhead related transcription factor 3; Forkhead-related protein FKHL7; Forkhead-related transcription factor 3; FOX C1; FOXC 1; FOXC1; FOXC1_HUMAN; FREAC 3; FREAC-3; FREAC3; homolog-like 7; IGDA; IHG 1; IHG1; IRID 1; IRID1; Iridogoniodysgenesis type 1; Myeloid factor delta.  
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  發育生物學  轉錄調節因子  表觀遺傳學  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 Human, Mouse, Rat,  (predicted: Chicken, Dog, Cow, Horse, )
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 57kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human FOXC1/FREAC3
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:
Binding of FREAC-3 and FREAC-4 to their cognate sites results in bending of the DNA at an angle of 80-90 degrees.
Involvement in disease; Defects in FOXC1 are the cause of Axenfeld-Rieger syndrome type 3 (RIEG3); also known as Axenfeld-Rieger syndrome (ARS) or Axenfeld syndrome or Axenfeld anomaly. It is characterized by posterior corneal embryotoxon, prominent Schwalbe line and iris adhesion to the Schwalbe line. Other features may be hypertelorism (wide spacing of the eyes), hypoplasia of the malar bones, congenital absence of some teeth and mental retardation. When associated with tooth anomalies, the disorder is known as Rieger syndrome. Glaucoma is a progressive blinding condition that occurs in approximately half of patients with Axenfeld-Rieger malformations.

Function:
Binding of FREAC-3 and FREAC-4 to their cognate sites results in bending of the DNA at an angle of 80-90 degrees.

Subunit:
Monomer.

Subcellular Location:
Nucleus.

Tissue Specificity:
Expressed in all tissues and cell lines examined.

DISEASE:
Defects in FOXC1 are the cause of iridogoniodysgenesis anomaly (IGDA) [MIM:601631]. IGDA is an autosomal dominant phenotype characterized by iris hypoplasia, goniodysgenesis, and juvenile glaucoma.
[DISEASE] Defects in FOXC1 are a cause of Peters anomaly (PAN) [MIM:604229]. Peters anomaly consists of a central corneal leukoma, absence of the posterior corneal stroma and Descemet membrane, and a variable degree of iris and lenticular attachments to the central aspect of the posterior cornea.


Similarity:
Contains 1 fork-head DNA-binding domain.

Database links:

Entrez Gene: 2296 Human

Entrez Gene: 17300 Mouse

GenBank: NP_001444 Human

Omim: 601090 Human

SwissProt: Q12948 Human

SwissProt: Q61572 Mouse

SwissProt: Q32NP8 Xenopus laevis

Unigene: 348883 Human

Unigene: 12949 Mouse



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