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(hào)           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
亚洲aⅴ,欧美一区二区精品国产激情爱,蜜桃做爰免费网站
Rabbit Anti-HPS1/HRP Conjugated antibody (bs-17379R-HRP)
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(jià)
產(chǎn)品編號(hào) bs-17379R-HRP
英文名稱 Rabbit Anti-HPS1/HRP Conjugated antibody
中文名稱 辣根過氧化物酶標(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(hào)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 WB=1:500-2000 ELISA=1:100-1000 IHC-P=1:50-200 IHC-F=1:50-200 ICC=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(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:
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(shí)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(duì)該病仍缺乏有效的治療辦法。通過對(duì)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(duì)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(hào): 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(hào): CQC24QY10047R0M/1100
京ICP備05066980號(hào)-1         京公網(wǎng)安備110107000727號(hào)
主站蜘蛛池模板: 在线观看av一区 | 国产精品亚洲一区 | a级黄色录像片 | 日日干夜夜弄 | 亚洲精品无码不卡在线播放 | 日本在线免费视频 | 成人免费久久网 | 91在线观看高清视频 | 久久99精品久久久秒播软件优势 | 日本一区二区在线视频 | 在线亚洲播放 | 国产成人亚洲精品另类动态 | 好紧好湿好黄的视频 | 亚洲欧美中文日韩v在线观看不卡 | 超碰96| 亚洲免费福利视频 | 人妻无码AV中文系列久久兔费 | 国内av在线 | 在线观看AV片永久免费 | 还珠格格第二部1至48集免费观看 | 乱子轮视频在线看 | 久草首页在线 | 亚洲在线中字幕 | 国产精品一区二区在线播放 | 国产精品久久久久久在线 | 久夜蜜汁av玖潮碰撩尤物 | 亚洲精品一区二区三区不 | 成片人免费观看一级A片 | 拔插拔插在线观看 | 成人性视频免费看的鲁片 | 国产亚洲中字幕欧 | 粗大黑人巨精大战欧美成人 | 国产成年人免费视频 | 精精精精xxxx免费视频 | 狠狠躁天天躁夜夜添人人 | 成人在线免费观看视频网站 | chinese白浆高潮videos | 一级高清 | 亚洲成年人 | 国产精品久久久久久久久免费相片 | 中文字幕乱码久久午夜 |