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Rabbit Anti-CORD2/OTX3/APC Conjugated antibody (bs-13990R-APC)
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產品編號 bs-13990R-APC
英文名稱 Rabbit Anti-CORD2/OTX3/APC Conjugated antibody
中文名稱 APC標記的視錐視桿相關蛋白CORD2抗體
別    名 Cone rod homeobox; Cone rod homeobox protein; Cone-rod homeobox protein; CORD 2; CRD; CRX; CRX_HUMAN; LCA 7; LCA7; Orthodenticle homeobox 3; OTX 3; OTX3.  
規(guī)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(jié)因子  表觀遺傳學  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 Rat,  (predicted: Human, Mouse, Dog, Cow, Horse, Sheep, Cat, )
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 32kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human CORD2
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 protein encoded by this gene is a photoreceptor-specific transcription factor which plays a role in the differentiation of photoreceptor cells. This homeodomain protein is necessary for the maintenance of normal cone and rod function. Mutations in this gene are associated with photoreceptor degeneration, Leber congenital amaurosis type III and the autosomal dominant cone-rod dystrophy 2. Several alternatively spliced transcript variants of this gene have been described, but the full-length nature of some variants has not been determined. [provided by RefSeq, Jul 2008]

Function:
Binds and transactivates the sequence 5'-TAATC[CA]-3' which is found upstream of several photoreceptor-specific genes, including the opsin genes. Acts synergistically with other transcription factors, e.g. NRL and RX, to regulate photoreceptor cell-specific gene transcription. Essential for the maintenance of mammalian photoreceptors.

Subcellular Location:
Nucleus.

Tissue Specificity:
Retina.

DISEASE:
Defects in CRX are the cause of Leber congenital amaurosis type 7 (LCA7) [MIM:613829]. LCA designates a clinically and genetically heterogeneous group of childhood retinal degenerations, generally inherited in an autosomal recessive manner. Affected infants have little or no retinal photoreceptor function as tested by electroretinography. LCA represents the most common genetic cause of congenital visual impairment in infants and children.
Defects in CRX are the cause of cone-rod dystrophy type 2 (CORD2) [MIM:120970]; also known as cone-rod retinal dystrophy 2 (CRD2). CORDs are inherited retinal dystrophies belonging to the group of pigmentary retinopathies. CORDs are characterized by retinal pigment deposits visible on fundus examination, predominantly in the macular region, and initial loss of cone photoreceptors followed by rod degeneration. This leads to decreased visual acuity and sensitivity in the central visual field, followed by loss of peripheral vision. Severe loss of vision occurs earlier than in retinitis pigmentosa.
Defects in CRX are a cause of retinitis pigmentosa (RP) [MIM:268000]. RP leads to degeneration of retinal photoreceptor cells. Patients typically have night vision blindness and loss of midperipheral visual field. As their condition progresses, they lose their far peripheral visual field and eventually central vision as well.

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

Database links:

Entrez Gene: 1406 Human

Entrez Gene: 280756 Cow

Entrez Gene: 12951 Mouse

Entrez Gene: 60446 Rat

Omim: 602225 Human

SwissProt: Q9XSK0 Cow

SwissProt: Q8SQ03 Dog

SwissProt: O43186 Human

SwissProt: O54751 Mouse

Unigene: 617342 Human

Unigene: 633434 Human

Unigene: 639114 Human

Unigene: 441911 Mouse

Unigene: 44287 Rat



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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