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

掃碼關注公眾號           掃碼咨詢技術支持           掃碼咨詢技術服務
  
客服熱線:400-901-9800  客服QQ:4009019800  技術答疑  技術支持  質量反饋  關于我們  聯系我們
国产精品久久国产精品99gif,国产乱子伦免费视频观看,人与性动交ⅩXXXB
首頁 > 產品中心 > 標記一抗 > 產品信息
Rabbit Anti-HPS1/PE Conjugated antibody (bs-17379R-PE)
訂購熱線:400-901-9800
訂購郵箱:sales@bioss.com.cn
訂購QQ:  400-901-9800
技術支持:techsupport@bioss.com.cn
說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產品編號 bs-17379R-PE
英文名稱 Rabbit Anti-HPS1/PE Conjugated antibody
中文名稱 PE標記的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.  
規格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領域 細胞生物  信號轉導  轉運蛋白  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 (predicted: Human, Mouse, Rat, Cow, Horse, )
產品應用 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 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,可導致出血時間延長、白化病、溶酶體膠質樣沉積等病狀。患者通常于30~50歲之間死于肺纖維化、出血、結腸炎等嚴重并發癥。目前對該病仍缺乏有效的治療辦法。通過對人、小鼠、酵母等的研究發現,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,轉運途徑受阻,表現為黑色素體、溶酶體、血小板致密體等多種亞細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一步闡明發病機制,也有利于建立一系列基因診斷和產前診斷方法,為將來的HPS基因治療提供理論依據。
版權所有 2004-2026 www.ggggww.cn 北京博奧森生物技術有限公司
通過國際質量管理體系ISO 9001:2015 GB/T 19001-2016    證書編號: 00124Q34771R2M/1100
通過國際醫療器械-質量管理體系ISO 13485:2016 GB/T 42061-2022    證書編號: CQC24QY10047R0M/1100
京ICP備05066980號-1         京公網安備110107000727號
主站蜘蛛池模板: 综合久久狠狠色成人网 | 日韩午夜精品免费理论片 | 99久久久久国产精品免费 | 99久久免费精品国产男女高不卡 | 99久久久久国产精品免费 | 亚洲欧美日韩综合久久久久久 | 欧美精品久久久久久久影视 | 性色av一区二区三区 | 日本免费一二三区中文 | 欧美性猛交xxxx乱大交久久 | 色猫咪av男人的天堂 | 成年人一级黄色片 | 热九九精品 | 国产精品国产三级国产普通话一 | 自拍偷拍亚洲欧美 | 亚洲天堂久久综合 | 一区二区三区四区不卡在线 | 国产免费特黄淫乱片 | 欧美成人一区二区 | 日韩在线一区视频 | 无遮无挡爽爽免费视频毛片韩国 | 极情综合网 | 日本不卡高字幕在线2019 | 国产高清在线精品一本大道 | 人妻系列无码专区免费视频 | 欧美美女一区二区 | 国产精品欧美久久久 | 欧美日韩一卡2卡三卡4卡乱码欧美孕交 | 日日干日日 | 强乱中文字幕亚洲精品 | 女同免费毛片在线播放 | 久久国产青偷人人妻潘金莲 | 久久99国产精品 | 久久精品国产精品亚洲色婷婷 | 日本不卡一区在线观看 | 丝袜a∨在线一区二区三区不卡 | 依依激情网| 国产精品久久偷人妻 | 欧美XXXX做受欧美69 | 亚洲免费一二三区 | 日韩欧美~中文字幕 |