| 產(chǎn)品編號 | bs-11689R-BF647 |
| 英文名稱(chēng) | Rabbit Anti-FGGY/BF647 Conjugated antibody |
| 中文名稱(chēng) | BF647標記的肌萎縮側索硬化癥相關(guān)蛋白FGGY抗體 |
| 別 名 | fggy; FGGY carbohydrate kinase domain containing; FGGY carbohydrate kinase domain-containing protein; FGGY_HUMAN; FLJ10986; MGC94804; OTTHUMP00000010078; OTTHUMP00000010081; OTTHUMP00000010082; OTTHUMP00000202071; RP11-242B9.1. |
| 規格價(jià)格 | 100ul/2980元 購買(mǎi) 大包裝/詢(xún)價(jià) |
| 說(shuō) 明 書(shū) | 100ul |
| 研究領(lǐng)域 | 細胞生物 神經(jīng)生物學(xué) 信號轉導 Alzheimer's |
| 抗體來(lái)源 | Rabbit |
| 克隆類(lèi)型 | Polyclonal |
| 交叉反應 | (predicted: Human, Mouse, Rat, Dog, Cow, Horse, Rabbit, Sheep, ) |
| 產(chǎn)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 | 60kDa |
| 性 狀 | Lyophilized or Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human FGGY (151-250aa) |
| 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(chǎn)品介紹 |
background: FGGY is a 551 amino acid member of the FGGY kinase family that exists as four isoforms which are produced by alternative splicing events. Expressed in lung, kidney, small intestine, liver and fetal brain, FGGY is encoded by a gene that maps to chromosome 1 and, when mutated, is associated with sporadic amyotrophic lateral sclerosis (ALS). ALS is a neurodegenerative disorder that affects motor neurons and results in fatal paralysis, usually within 2 to 5 years after initial diagnosis. Chromosome 1, on which the gene encoding FGGY is located, is the largest human chromosome, spanning about 260 million base pairs and making up 8% of the human genome. There are about 3,000 genes on chromosome 1, many of which are associated with genetic diseases, including Hutchinson-Gilford progeria, familial adenomatous polyposis, Stickler syndrome, Gaucher disease and Usher syndrome. Function: Expressed in kidney, lung and small intestine and to a lower extent in liver and detected in cerebrospinal fluid (at protein level). Tissue Specificity: Expressed in fetal brain (at protein level). DISEASE: Defects in FGGY are associated with sporadic amyotrophic lateral sclerosis (ALS) [MIM:105400]. Amyotrophic lateral sclerosis is a neurodegenerative disorder affecting upper and lower motor neurons and resulting in fatal paralysis. Sensory abnormalities are absent. Death usually occurs within 2 to 5 years. The etiology of amyotrophic lateral sclerosis is likely to be multifactorial, involving both genetic and environmental factors. Similarity: Belongs to the FGGY kinase family. Database links: UniProtKB/Swiss-Prot: Q96C11.2 Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
