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CORD2/OTX3 Rabbit pAb (bs-13990R)  
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50ul/1180.00元
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产品编号 bs-13990R
英文名称 CORD2/OTX3 Rabbit pAb
中文名称 视锥视杆相关蛋白CORD2抗体
别    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.  
研究领域 神经生物学  转录调节因子  表观遗传学  
抗体来源 Rabbit
克隆类型 Polyclonal
克 隆 号
交叉反应 Rat (predicted: Human,Mouse,Sheep,Cow,Dog,Cat,Horse)
产品应用 IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理论分子量 32 kDa
检测分子量
细胞定位 细胞核 
性    状 Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human CORD2: 1-100/299 
亚    型 IgG
纯化方法 affinity purified by Protein A
缓 冲 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存条件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事项 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
产品介绍 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.

SWISS:
O43186

Gene ID:
1406

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



产品图片
Tissue/cell: rat brain tissue; 4% Paraformaldehyde-fixed and paraffin-embedded; Antigen retrieval: citrate buffer ( 0.01M, pH 6.0 ), Boiling bathing for 15min; Block endogenous peroxidase by 3% Hydrogen peroxide for 30min; Blocking buffer (normal goat serum,C-0005) at 37℃ for 20 min; Incubation: Anti-CORD2/OTX3 Polyclonal Antibody, Unconjugated(bs-13990R) 1:200, overnight at 4°C, followed by conjugation to the secondary antibody(SP-0023) and DAB(C-0010) staining
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