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Rabbit Anti-RAXL1/BF647 Conjugated antibody (bs-21124R-BF647)
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-21124R-BF647
英文名稱 Rabbit Anti-RAXL1/BF647 Conjugated antibody
中文名稱 BF647標記的視網膜前神經折疊同源框蛋白抗體
別    名 ARMD6; CORD11; Macular degeneration, age related 6, included; MGC15631; Q50 type retinal homeobox; Q50-type retinal homeobox protein; QRX; rax2; RAX2_HUMAN; RAXL1; Retina and anterior neural fold homeobox like 1; Retina and anterior neural fold homeobox protein 2; Retina and anterior neural fold homeobox-like protein 1.  
規格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領域 神經生物學  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 (predicted: Human, Chimpanzee, )
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 21kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human RAXL1
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 encodes a homeodomain-containing protein that plays a role in eye development. Mutation of this gene causes age-related macular degeneration type 6, an eye disorder resulting in accumulations of protein and lipid beneath the retinal pigment epithelium and within the Bruch's membrane. Defects in this gene can also cause cone-rod dystrophy type 11, a disease characterized by the initial degeneration of cone photoreceptor cells and resulting in loss of color vision and visual acuity, followed by the degeneration of rod photoreceptor cells, which progresses to night blindness and the loss of peripheral vision. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Jan 2016]

Function:
May be involved in modulating the expression of photoreceptor specific genes. Binds to the Ret-1 and Bat-1 element within the rhodopsin promoter.

Subcellular Location:
Nucleus.

DISEASE:
Defects in RAX2 are the cause of age-related macular degeneration type 6 (ARMD6) [MIM:613757]. ARMD is in most patients manifest as ophthalmoscopically visible yellowish accumulations of protein and lipid (known as drusen) that lie beneath the retinal pigment epithelium and within an elastin-containing structure known as Bruch's membrane. ARMD is likely to be a mechanistically heterogeneous group of disorders. Defects in RAX2 are the cause of cone-rod dystrophy type 11 (CORD11)
[MIM:610381]. CORD is characterized by the initial degeneration of cone photoreceptor cells, thus causing early loss of visual acuity and color vision, followed by the degeneration of rod photoreceptor cells and leading to progressive night blindness and peripheral visual field loss.

Similarity:
Contains 1 homeobox DNA-binding domain.

Database links:

Entrez Gene: 84839 Human

Omim: 610362 Human

SwissProt: Q96IS3 Human

Unigene: 532691 Human



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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