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Rabbit Anti-CNGB1/APC Conjugated antibody (bs-11332R-APC)
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說(shuō) 明 書(shū): 100ul  
100ul/2980.00元
大包裝/詢(xún)價(jià)
產(chǎn)品編號(hào) bs-11332R-APC
英文名稱(chēng)1 Rabbit Anti-CNGB1/APC Conjugated antibody
中文名稱(chēng) APC標(biāo)記的環(huán)核苷酸門(mén)控陽(yáng)離子通道蛋白CNG-1β抗體
別    名 CNCG 4; CNCG2; CNCG3L; CNCG4; 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; GARP; GARP2; Glutamic acid rich protein; RCNC2; RCNCb; RCNCbeta; RP45; 240 kDa protein of rod photoreceptor CNG channel; cGMP gated cation channel beta subunit; Truncated glutamic acid rich protein; Truncated rod photoreceptor cGMP gated channel beta subunit; Truncated rod photoreceptor cGMP-gated channel beta-subunit; CNGB1_HUMAN.  
規(guī)格價(jià)格 100ul/2980元 購(gòu)買(mǎi)        大包裝/詢(xún)價(jià)
說(shuō) 明 書(shū) 100ul  
研究領(lǐng)域 細(xì)胞生物  神經(jīng)生物學(xué)  干細(xì)胞  細(xì)胞粘附分子  細(xì)胞骨架  細(xì)胞外基質(zhì)  
抗體來(lái)源 Rabbit
克隆類(lèi)型 Polyclonal
交叉反應(yīng) (predicted: Human, Mouse, Rat, Pig, Cow, Horse, Rabbit, Sheep, )
產(chǎn)品應(yīng)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
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human truncated GARP
亞    型 IgG
純化方法 affinity purified by Protein A
儲(chǔ) 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(chǎn)品介紹 background:
Glutamic acid rich protein (GARP) is a soluble protein localized to the outer segments of the rod photoreceptor. It forms 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. There are 3 isoforms produced by alternative splicing. Isoform GARP2 is a high affinity rod photoreceptor phosphodiesterase (PDE6)-binding protein that modulates its catalytic properties; it is a regulator of spontaneous activation of rod PDE6, thereby serving to lower rod photoreceptor 'dark noise' and allowing these sensory cells to operate at the single photon detection limit. Defects in GARP are the cause of retinitis pigmentosa type 25 (RP25). 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.

Function:
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.
Isoform GARP2 is a high affinity rod photoreceptor phosphodiesterase (PDE6)-binding protein that modulates its catalytic properties: it is a regulator of spontaneous activation of rod PDE6, thereby serving to lower rod photoreceptor 'dark noise' and allowing these sensory cells to operate at the single photon detection limit.

Subunit:
Heterooligomeric complex with CNGA1.

Subcellular Location:
Membrane; Multi-pass membrane protein.

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.

Database links:

Entrez Gene: 1258 Human

Entrez Gene: 333329 Mouse

Entrez Gene: 83686 Rat

Omim: 600724 Human

SwissProt: Q14028 Human

SwissProt: 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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