超碰97自拍_久久99久久98精品免观看软件_在线亚洲一区_九九视频这里只有精品_国产精品一区二区欧美视频_精品国产区一区

掃碼關(guān)注公眾號           掃碼咨詢技術(shù)支持           掃碼咨詢技術(shù)服務(wù)
  
客服熱線:400-901-9800  客服QQ:4009019800  技術(shù)答疑  技術(shù)支持  質(zhì)量反饋  關(guān)于我們  聯(lián)系我們
微拍福利88,av在线资源观看,久草手机在线观看视频
Rabbit Anti-HPS1/Cy3 Conjugated antibody (bs-17379R-Cy3)
訂購熱線:400-901-9800
訂購郵箱:sales@bioss.com.cn
訂購QQ:  400-901-9800
技術(shù)支持:techsupport@bioss.com.cn
說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產(chǎn)品編號 bs-17379R-Cy3
英文名稱 Rabbit Anti-HPS1/Cy3 Conjugated antibody
中文名稱 Cy3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)格價格 100ul/2980元 購買        大包裝/詢價
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 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 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

Entrez Gene: 114638 Rat

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)致出血時間延長、白化病、溶酶體膠質(zhì)樣沉積等病狀。患者通常于30~50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)。
版權(quán)所有 2004-2026 www.ggggww.cn 北京博奧森生物技術(shù)有限公司
通過國際質(zhì)量管理體系ISO 9001:2015 GB/T 19001-2016    證書編號: 00124Q34771R2M/1100
通過國際醫(yī)療器械-質(zhì)量管理體系ISO 13485:2016 GB/T 42061-2022    證書編號: CQC24QY10047R0M/1100
京ICP備05066980號-1         京公網(wǎng)安備110107000727號
主站蜘蛛池模板: 草青青视频 | 国产精品精品久久久 | 国产资源免费观看 | 国产香蕉97碰碰久久人人九色 | 欧美国内亚洲 | 亚洲av中文无码乱人伦在线播放 | 日本一区二区色 | 无码日韩人妻AV一区免费 | 在线观看免费视频亚洲 | 精品亚洲aⅴ一区二区三区 深夜激情视频 | 特级毛片在线 | 在线观看自拍 | 亚洲精品国产精华 | 久久亚洲精品成人av | av有码在线 | a级毛毛片| 丝袜国偷自产中文字幕亚洲第一页 | 久久成人在线观看 | 国产无遮挡又黄又爽动态图 | 毛片毛片毛片毛片毛片毛片 | 五十路熟妇强烈无码 | 久久久精品区 | 粉嫩一区二区性色av97 | 人妻无码中文字幕一区二区三区 | 欧美成人手机视频 | 亚洲成人黄色网址 | 日本久草 | 日本一区二区在线视频 | 亚洲伊人久久精品影院一美女洗澡 | 日本免费一二三区中文 | 超碰av网站 | 在线中文字幕网 | 久久久久久久高清 | 欧美毛茸茸 | 亚洲国产精品久久久久婷婷老年 | 国产精品爽爽爽 | 国产精品JⅠZZ视频 av解说在线精品 | 92少妇午夜福利视频在线 | 超碰caoporm97 | 国产一级特黄真人毛片 | 一级毛片中国 |