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Rabbit Anti-SAR1B/BF594 Conjugated antibody (bs-19392R-BF594)
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價(jià)
產(chǎn)品編號(hào) bs-19392R-BF594
英文名稱1 Rabbit Anti-SAR1B/BF594 Conjugated antibody
中文名稱 BF594標(biāo)記的GTP結(jié)合蛋白SAR1B抗體
別    名 ANDD; CMRD; GTBPB; GTP binding protein SAR1b; GTP binding protein Sara; GTP-binding protein B; GTP-binding protein SAR1B; SAR1 gene homolog B (S. cerevisiae); SAR1 homolog B; SAR1a gene homolog 2; SAR1B; SAR1B_HUMAN; SARA2; SARB.  
規(guī)格價(jià)格 100ul/2980元 購(gòu)買        大包裝/詢價(jià)
說 明 書 100ul  
研究領(lǐng)域 細(xì)胞生物  信號(hào)轉(zhuǎn)導(dǎo)  結(jié)合蛋白  G蛋白信號(hào)  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng)
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 22kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human SAR1B
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:
The protein encoded by this gene is a small GTPase that acts as a homodimer. The encoded protein is activated by the guanine nucleotide exchange factor PREB and is involved in protein transport from the endoplasmic reticulum to the Golgi. This protein is part of the COPII coat complex. Defects in this gene are a cause of chylomicron retention disease (CMRD), also known as Anderson disease (ANDD). Two transcript variants encoding the same protein have been found for this gene. [provided by RefSeq, Mar 2010]

Function:
Involved in transport from the endoplasmic reticulum to the Golgi apparatus. Activated by the guanine nucleotide exchange factor PREB. Involved in the selection of the protein cargo and the assembly of the COPII coat complex.

Subunit:
Homodimer. Binds PREB. Part of the COPII coat complex. Binds to the cytoplasmic tails of target proteins in the endoplasmic reticulum (By similarity).

Subcellular Location:
Endoplasmic reticulum membrane. Golgi apparatus > Golgi stack membrane. Associated with the endoplasmic reticulum and Golgi stacks, in particular in the juxta-nuclear Golgi region.

Tissue Specificity:
Expressed in many tissues including small intestine, liver, muscle and brain.

DISEASE:
Defects in SAR1B are the cause of chylomicron retention disease (CMRD) [MIM:246700]; also known as Anderson disease (ANDD). CMRD is an autosomal recessive disorder of severe fat malabsorption associated with failure to thrive in infancy. The condition is characterized by deficiency of fat-soluble vitamins, low blood cholesterol levels, and a selective absence of chylomicrons from blood. Affected individuals accumulate chylomicron-like particles in membrane-bound compartments of enterocytes, which contain large cytosolic lipid droplets.

Similarity:
Belongs to the small GTPase superfamily. SAR1 family.

Database links:

Entrez Gene: 51128 Human

Entrez Gene: 416314 Chicken

Entrez Gene: 515999 Cow

Entrez Gene: 66397 Mouse

Entrez Gene: 494017 Pig

Entrez Gene: 287276 Rat

Omim: 607690 Human

SwissProt: Q3T0T7 Cow

SwissProt: Q9Y6B6 Human

SwissProt: Q9CQC9 Mouse

SwissProt: Q5PYH3 Pig

SwissProt: Q5HZY2 Rat

Unigene: 432984 Human

Unigene: 196592 Mouse

Unigene: 36748 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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