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
china70years老女人,日本不卡视频,久久日韩视频
Rabbit Anti-HPS1/PE-Cy5.5 Conjugated antibody (bs-17379R-PE-Cy5.5)
訂購(gòu)熱線:400-901-9800
訂購(gòu)郵箱:sales@bioss.com.cn
訂購(gòu)QQ:  400-901-9800
技術(shù)支持:techsupport@bioss.com.cn
說(shuō) 明 書: 100ul  
100ul/2980.00元
大包裝/詢價(jià)
產(chǎn)品編號(hào) bs-17379R-PE-Cy5.5
英文名稱 Rabbit Anti-HPS1/PE-Cy5.5 Conjugated antibody
中文名稱 PE-Cy5.5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(gòu)買        大包裝/詢價(jià)
說(shuō) 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(lái)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(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(jiǎn)稱HPS),是常染色體隱性遺傳病,可導(dǎo)致出血時(shí)間延長(zhǎng)、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(guò)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(lái)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(guò)國(guó)際質(zhì)量管理體系ISO 9001:2015 GB/T 19001-2016    證書編號(hào): 00124Q34771R2M/1100
通過(guò)國(guó)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: 一区二区三区 | 后入内射欧美99二区视频 | 亚洲高清精品中出 | 免费看的av网站 | 福利社一区| 黄色va视频 | 国产美女极度色诱视频WWW | 久久艹伊人 | 成全视频在线观看免费高清在线观看 | 岛国无码av不卡一区二区 | 亚洲天堂福利视频 | 后入白嫩网红内射99XYZ | 久久嫩草久久久精品三区 | BT天堂新版中文在线 | 无码人妻丰满熟妇啪啪区日韩久久 | 无遮挡3d黄肉动漫午夜 | 亚洲天堂福利视频 | 天天看天天摸 | 男的操女的免费视频 | 岛国无码av不卡一区二区 | 少妇高潮水多太爽了动态图 | 亚洲欧美日韩国产成人一区 | 亚洲欧美一区在线观看 | 中国一极毛片 | 亚洲av永久无码精品国产精品 | 成年人免费视频网站 | 欧美亚洲另类丝袜综合网 | 亚洲一区二区三区尿失禁 | 亚洲AV综合色区手机无码一区 | 亚州激情| 在线观看成人免费视频 | 亚洲精品国产成人 | 国产精品视频二区三区 | 日本在线不卡视频一二三区 | 美女不带套日出白浆免费视频 | 狠狠插久久综合网最新章节 | 他掀开裙子舌头伸进去在线观看 | 免费a级毛片无码免费视频120软件 | 40岁成熟女人牲交片 | 青青小草AV一区二区三区 | 成人在线免费视频 |