產(chǎn)品編號 | bs-17379R-Cy7 |
英文名稱 | Rabbit Anti-HPS1/Cy7 Conjugated antibody |
中文名稱 | Cy7標(biāo)記的Hermansky-Pudlak綜合征蛋白1抗體 |
別 名 | Hermansky Pudlak syndrome 1 protein; Hermansky Pudlak syndrome gene; Hermansky Pudlak syndrome type 1; Hermansky-Pudlak syndrome 1; Hermansky-Pudlak syndrome 1 protein; HPS; HPS1; HPS1_HUMAN; MGC5277. |
規(guī)格價(jià)格 | 100ul/2980元 購買 大包裝/詢價(jià) |
說 明 書 | 100ul |
研究領(lǐng)域 | 細(xì)胞生物 信號轉(zhuǎn)導(dǎo) 轉(zhuǎn)運(yùn)蛋白 |
抗體來源 | Rabbit |
克隆類型 | Polyclonal |
交叉反應(yīng) | (predicted: Human, Mouse, Rat, Cow, Horse, ) |
產(chǎn)品應(yīng)用 |
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
分 子 量 | 79kDa |
性 狀 | Lyophilized or Liquid |
濃 度 | 1mg/ml |
免 疫 原 | KLH conjugated synthetic peptide derived from human HPS1 |
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: This gene encodes a protein that may play a role in organelle biogenesis associated with melanosomes, platelet dense granules, and lysosomes. The encoded protein is a component of three different protein complexes termed biogenesis of lysosome-related organelles complex (BLOC)-3, BLOC4, and BLOC5. Mutations in this gene are associated with Hermansky-Pudlak syndrome type 1. Multiple transcript variants encoding distinct isoforms have been identified for this gene; the full-length sequences of some of these have not been determined yet. [provided by RefSeq, Jul 2008] Function: Component of multiple cytoplasmic organelles. Apparently crucial for their normal development and function. May be involved in intracellular protein sorting. Tissue Specificity: Ubiquitous. DISEASE: Defects in HPS1 are the cause of Hermansky-Pudlak syndrome type 1 (HPS1) [MIM:203300]. Hermansky-Pudlak syndrome (HPS) is a genetically heterogeneous, rare, autosomal recessive disorder characterized by oculocutaneous albinism, bleeding due to platelet storage pool deficiency, and lysosomal storage defects. This syndrome results from defects of diverse cytoplasmic organelles including melanosomes, platelet dense granules and lysosomes. Ceroid storage in the lungs is associated with pulmonary fibrosis, a common cause of premature death in individuals with HPS. Database links: Entrez Gene: 3257 Human Entrez Gene: 192236 Mouse Omim: 604982 Human SwissProt: Q92902 Human SwissProt: O08983 Mouse Unigene: 404568 Human Unigene: 218381 Mouse Unigene: 17691 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. Hermansky-Pudlak綜合征(簡稱HPS),是常染色體隱性遺傳病,可導(dǎo)致出血時(shí)間延長、白化病、溶酶體膠質(zhì)樣沉積等病狀?;颊咄ǔS?0~50歲之間死于肺纖維化、出血、結(jié)腸炎等嚴(yán)重并發(fā)癥。目前對該病仍缺乏有效的治療辦法。通過對人、小鼠、酵母等的研究發(fā)現(xiàn),這是一種單基因病,但涉及到多個(gè)不同基因的突變。基因突變后,轉(zhuǎn)運(yùn)途徑受阻,表現(xiàn)為黑色素體、溶酶體、血小板致密體等多種亞細(xì)胞器的生物合成或功能同時(shí)受累的病理改變。對這些HPS基因的克隆既有利于進(jìn)一步闡明發(fā)病機(jī)制,也有利于建立一系列基因診斷和產(chǎn)前診斷方法,為將來的HPS基因治療提供理論依據(jù)。 |
| 国产又粗又猛又黄又爽无遮挡 | 欧美一区二区三区在线视频 | ThePorn精品无码 | 国产亚洲无码视频 | 免费观看一级毛一片 | 日韩人妻精品中文字幕 | 农村乱视频一区二区三区 | 91国内精品久久久久夏晴子 | 自拍视频在线观看 | 插插女免费视频疯狂 | 国产婬乱寡妇A毛片 | 国产片一区二区三区 | 久久偷看各类wc女厕嘘嘘污黄 | 粉嫩av精色欲人妻精品 | 国产乱妇乱子在线播放视频国产 | 柚子TV成人免费视频网站 | 欧美群交性XXXX狂野 | 开心网五月天中文字幕 | 国产熟妇婬乱A片免费看 | 久久精品www人人爽人人 | 一区二区中文字幕 | 91久久精品无码一区二区三区 | 欧美午夜在线观看 | 精品人妻一区二区三区线国色天 | 免费在线中文字幕 | 99久久婷婷国产一区二区三区 | 亂倫近親相姦免费中文字幕 | 麻豆国产91 在线播放 | 成人免费A片在线观看直播96 | 北京熟妇槡BBBB槡BBBB | 日本高清视频在线播放 | 紧身裤蜜桃臀久久影院 | 中文字幕乱偷在线影院 | 寡妇2高潮一级毛片 | 国产视频无码在线观看 | 免费在线观看午夜福利 | 嫩草一区二区国产乱码99人妻 | 日本黑人乱偷人妻中文字幕 | 91无码人妻精品1国产 | 自拍日韩亚洲一区在线 |