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Rabbit Anti-HPS1/BF594 Conjugated antibody (bs-17379R-BF594)
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產品編號 bs-17379R-BF594
英文名稱 Rabbit Anti-HPS1/BF594 Conjugated antibody
中文名稱 BF594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
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。
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