產(chǎn)品編號(hào) | bs-13922R-BF555 |
英文名稱(chēng)1 | Rabbit Anti-Chorein/BF555 Conjugated antibody |
中文名稱(chēng) | BF555標(biāo)記的液泡蛋白分選蛋白VPS13A抗體 |
別 名 | CHAC; Chorea acanthocytosis; Chorea acanthocytosis protein; FLJ42030; KIAA0986; Vacuolar protein sorting 13 homolog A (S. cerevisiae); Vacuolar protein sorting 13 homolog A; Vacuolar protein sorting 13A; Vacuolar protein sorting associated protein 13A; VPS 13A; VP13A_HUMAN; VPS13A. |
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) 信號(hào)轉(zhuǎn)導(dǎo) |
抗體來(lái)源 | Rabbit |
克隆類(lèi)型 | Polyclonal |
交叉反應(yīng) | (predicted: Human, Mouse, Rat, Cow, Horse, 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 | 360kDa |
性 狀 | Lyophilized or Liquid |
濃 度 | 1mg/ml |
免 疫 原 | KLH conjugated synthetic peptide derived from human Chorein |
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: Chorein may control steps in the cycling of proteins through the trans-Golgi network to endosomes, lysosomes and the plasma membrane. Mutations in this gene cause the autosomal recessive disorder chorea-acanthocytosis. Alternative splicing of this gene results in multiple transcript variants. Function: May play a role in the control of protein cycling through the trans-Golgi network to early and late endosomes, lysosomes and plasma membrane. Tissue Specificity: Widely expressed. Higher expression is found in brain, heart, skeletal muscle and kidney. DISEASE: Choreoacanthocytosis (CHAC) [MIM:200150]: An autosomal recessive neurodegenerative disorder characterized by the gradual onset of hyperkinetic movements and abnormal erythrocyte morphology. Basal ganglia atrophy in the brain is a pathological feature of the disease. Other clinical symptoms include psychiatric features, epilepsy, peripheral neuropathy, myopathy and oral self-mutilation. Note=The disease is caused by mutations affecting the gene represented in this entry. Similarity: Belongs to the VPS13 family. Contains 10 TPR repeats. Database links: Entrez Gene: 23230 Human Omim: 605978 Human SwissProt: Q96RL7 Human Unigene: 459790 Human Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |