次甲基綠號(hào)：224967-52-6   
英文名稱：Methylene green；Methylene Green zinc chloride double salt；Basic Green 5；C.I. 52020  
其他名稱：堿性綠5；亞甲基綠   
號(hào)：224967-52-6    
C16H17ClN4O2S?0.5ZnCl2=433.0   
級(jí)別：For microscopy   
含量：≥80.0%   
性狀(以下信息僅供參考)：暗綠色或褐色或黑色粉末，溶于水   
用途：本品僅供科研，不得用于其它用途。   
保存：RT次甲基綠號(hào)：224967-52-6儲(chǔ)存條件：
避光、干燥陰涼處封閉貯存,嚴(yán)禁與有毒、有害物品混放、混運(yùn)。本品為非危險(xiǎn) 產(chǎn)品可按一般化學(xué)品運(yùn)輸,輕搬動(dòng)輕放,防止日曬、雨淋！受熱、受潮、受光后易喪失活力,保存期短,因此貯存和運(yùn)輸條件比較苛刻。
運(yùn)輸：汽車運(yùn)輸、EMS郵政快遞，申通快遞等， 款到上海3天內(nèi)發(fā)貨； 
售后：如您對(duì)我們的產(chǎn)品服務(wù)及技術(shù)指標(biāo)有特殊要求，請(qǐng)及時(shí)通知我方。 
存儲(chǔ)：應(yīng)貯存在干燥清潔避光的環(huán)境中，嚴(yán)禁與有毒物質(zhì)混放，以免污染（保質(zhì)期為兩年）。 
次甲基綠號(hào)：224967-52-6主要優(yōu)級(jí)純、分級(jí)純和化學(xué)純3種：
（1）優(yōu)級(jí)純（GR：Guaranteed reagent），又稱一級(jí)品或保證試劑，99.8%，這種試劑純度Z高，雜質(zhì)含量Z低，適合于重要精密的分析工作和科學(xué)研究工作，使用綠色瓶簽。
（2）分析純（AR），又稱二級(jí)試劑，純度很高，99.7%，略次于優(yōu)級(jí)純，適合于重要分析及一般研究工作，使用紅色瓶簽。
（3）化學(xué)純（CP），又稱三級(jí)試劑，≥ 99.5%，純度與分析純相差較大，適用于工礦、學(xué)校一般分析工作。使用藍(lán)色（深藍(lán)色）標(biāo)簽。
（4）實(shí)驗(yàn)試劑（LR：Laboratory reagent），又稱四級(jí)試劑。
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NDUFS7 are a cause of mitochondrial complex I deficiency (MT-C1D) [MIM:252010]. A disorder of the mitochondrial respiratory chain that causes a wide range of clinical disorders, from lethal neonatal disease to adult-onset neurodegenerative disorders. Phenotypes include macrocephaly with progressive leukodystrophy, non-specific encephalopathy, cardiomyopathy, myopathy, liver disease, Leigh syndrome, Leber hereditary optic neuropathy, and some forms of Parkinson disease.
Similarity : Belongs to the complex I 20 kDa subunit family.
Database links : UniProtKB/Swiss-Prot: O75251.3
英文名稱  Anti-NPC2/Niemann Pick C2
中文名稱  尼曼匹克C2前體蛋白抗體
別    名  EDDM1; Epididymal protein 1; Epididymal secretory protein; Epididymal secretory protein E1; HE1; Human epididymis-specific protein 1; Niemann-Pick disease type C2; Niemann-Pick disease type C2 protein; NPC2; NPC2_HUMAN; Tissue specific secretory protein.
濃    度  1mg/1ml
規(guī) 格  0.2ml/200μg
抗體來源  Rabbit 
克隆類型  polyclonal
次甲基綠號(hào)：224967-52-6交叉反應(yīng)  Human, Mouse, Rat, Cow, Sheep
產(chǎn)品類型  一抗   
研究領(lǐng)域  腫瘤 心血管 細(xì)胞生物 神經(jīng)生物學(xué) 信號(hào)轉(zhuǎn)導(dǎo) 
蛋白分子量  predicted molecular weight: 15kDa
性    狀  Lyophilized or Liquid
免 疫 原  KLH conjugated synthetic peptide derived from human Niemann Pick C2 (20-80aa)
亞    型  IgG
純化方法  affinity purified by Protein A
儲(chǔ) 存 液  Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4
產(chǎn)品應(yīng)用   WB=1:100-500  ELISA=1:500-1000  IHC-P=1:100-500  IHC-F=1:100-500  ICC=1:100-500  IF=1:100-500
（石蠟切片需做抗原修復(fù)）
 not yet tested in other applications.
 optimal dilutions/concentrations should be determined by the end user. 
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.
Important Note  This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
產(chǎn)品介紹 NPC2 is a secreted protein mapping against gene 14q24.3 (1,2). NPC2 regulates the lipid composition of sperm membranes during maturation in the epididymis(1,2). Mutations in the NPC2 gene may cause Nieman-Pick type C2 disease and frontal lobe atrophy (1,2,3). Nieman-Pick type C2 is a fatal hereditary disease characterized by defective lysosome release of cholesterol (3). The disease is caused by HE1 deficiency, a lysosmal protein proven to be undetectable in fibroblasts from NPC2 patients (3). This differentiates NPC2 from NPC1, as NPC1 has HE1 protein present (3).
Function : May be involved in the regulation of the lipid composition of sperm membranes during the maturation in the epididymis.
Subunit : Interacts with NUS1/NgBR, the interaction stabilizes NCP2 and regulates cholesterol trafficking. Interacts with DHDDS. Interacts with NPC1 (via the second lumenal domain) in a cholestrol-dependent manner (By similarity). Interacts with NEDD4L (via C2 domain) (By similarity). Interacts with NPC1L1.
Subcellular Location : Secreted. Endoplasmic reticulum. Lysosome.
Tissue Specificity : Epididymis.
DISEASE : Defects in NPC2 are the cause of Niemann-Pick disease type C2 (NPDC2) [MIM:607625]. A lysosomal storage disorder that affects the viscera and the central nervous system. It is due to defective intracellular processing and transport of low-density lipoprotein derived cholesterol. It causes accumulation of cholesterol in lysosomes, with delayed induction of cholesterol homeostatic reactions. Niemann-Pick disease type C2 has a