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Rabbit Anti-GARP/FITC Conjugated antibody (bs-12093R-FITC)
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產品編號 bs-12093R-FITC
英文名稱1 Rabbit Anti-GARP/FITC Conjugated antibody
中文名稱 FITC標記的環磷酸鳥苷門控通道蛋白CNG4抗體
別    名 CNCG2; CNCG3L; CNCG4; CNG 4; CNG-4; CNG channel 4; CNG channel beta 1; CNG4; CNGB 1; CNGB1; CNGB1B; Cyclic nucleotide gated cation channel 4; Cyclic nucleotide gated cation channel; Cyclic nucleotide gated cation channel beta 1; Cyclic nucleotide gated cation channel gamma; Cyclic nucleotide gated cation channel modulatory subunit; Cyclic nucleotide gated channel (photoreceptor) cGMP gated 3 (gamma) like; Cyclic nucleotide gated channel beta 1; GAR1; Glutamic acid rich protein; RCNC2; RCNCb; RCNCbeta; RP45; CNGB1_HUMAN.  
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  通道蛋白  細胞膜受體  G蛋白偶聯受體  G蛋白信號  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 (predicted: Human, Mouse, Rat, Pig, Cow, Horse, 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 140kDa
細胞定位 細胞膜 
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human CNG4
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:
Cyclic nucleotide-gated (CNG) cation channels are heteromeric complexes made up of principal alpha and modulatory beta subunits (1,2). The alpha subunits consist of CNG1-3 and form functional cation channels by themselves (1,2). The beta subunits consist of CNG4-6 and, unlike the alpha subunits, do not form functional channels, but rather modify the properties of channels (1,2). CNG channels are essential components of olfactory and visual transduction (1,2). In olfactory neurons, CNG2, CNG4.3 and CNG5 form Ca2+ permeable channels, which open and depolarize the cell in response to cAMP (1-3). In rod photoreceptors, CNG1 and CNG4.1 combine to form Ca ion permeable channels, which give rise to a current in response to cGMP (1-3). CNG3 and CNG6 are expressed in cone receptors and may combine to form a native cGMP-activated channel (2,3). CNG channels have been implicated in other areas (4-6). CNG1 is also expressed in medium-sized and small-sized arteries, suggesting a role for CNG in the regulation of arterial blood pressure and of blood supply to different regions (4). CNG1, CNG4.1 and CNG4.2 have been detected in the rat pineal gland (5). CNG2, CNG4.3 and CNG5 are present in GT1 cell lines and may play a role in the secretion of gonadotropin-releasing hormone (6).

Function:
GARP is a subunit of cyclic nucleotide-gated (CNG) channels, nonselective cation channels, which play important roles in both visual and olfactory signal transduction. When associated with CNGA1, it is involved in the regulation of ion flow into the rod photoreceptor outer segment (ROS), in response to light-induced alteration of the levels of intracellular cGMP. Defects in GARP are the cause of retinitis pigmentosa type 45 (RP45). 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.

Subunit:
Heterooligomeric complex with CNGA1.

Subcellular Location:
Membrane; Multi-pass membrane protein.

Tissue Specificity:
Prominently expressed in retina.

DISEASE:
Defects in CNGB1 are the cause of retinitis pigmentosa type 45 (RP45) [MIM:613767]. 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 cyclic nucleotide-gated cation channel (TC 1.A.1.5) family. CNGB1 subfamily.
Contains 1 cyclic nucleotide-binding domain.

Database links:

Entrez Gene: 1258 Human

Entrez Gene: 333329 Mouse

Entrez Gene: 83686 Rat

Omim: 600724 Human

SwissProt: Q14028 Human

  • wissProt: O35788 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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