[{"data":1,"prerenderedAt":-1},["ShallowReactive",2],{"currency-rates":3,"$fyk2oeqxpb7bd":8,"compound-J-1978433-5":171,"verified-suppliers-J-1978433-5":353},{"rates":4,"source":7},{"USD":5,"CNY":6},1,6.689327,"live",{"rows":9,"total":170,"current":5,"size":145},[10,33,45,60,74,85,96,113,123,135,146,158],{"slug":11,"cid":12,"imgSvg":13,"name":14,"nameZh":15,"cas":16,"formula":17,"iupac":14,"category":18,"categoryName":19,"categoryEnName":20,"industry":21,"tags":22,"offersCount":24,"supplierCount":25,"bestOffer":26},"843627791009189888","J-1971488-8","\u002Fimages\u002Fchem_structures_svg\u002F399\u002F65845-61-6.svg","N-Methylcyclohexanamine hydrochloride","N-甲基环己胺盐酸盐","65845-61-6","C7H16ClN","acids","有机酸","Organic Acids","",[23],"In Stock",4,2,{"unitPrice":27,"pack":28,"currency":29,"origin":30,"originFlag":31,"moq":21,"stock":32},2.64,"500g","CNY","China","CN","in-stock",{"slug":34,"cid":35,"imgSvg":36,"name":37,"nameZh":38,"cas":39,"formula":40,"iupac":37,"category":18,"categoryName":19,"categoryEnName":20,"industry":21,"tags":41,"offersCount":24,"supplierCount":5,"bestOffer":42},"785882541994283008","J-1323843-4","\u002Fimages\u002Fchem_structures_svg\u002F104\u002F67627-18-3.svg","Potassium indigotrisulfonate","靛蓝三磺酸钾","67627-18-3","C16H7K3N2O11S3",[23],{"unitPrice":43,"pack":44,"currency":29,"origin":30,"originFlag":31,"moq":21,"stock":32},180,"5g",{"slug":46,"cid":47,"imgSvg":48,"name":49,"nameZh":50,"cas":51,"formula":52,"iupac":49,"category":53,"categoryName":54,"categoryEnName":55,"industry":21,"tags":56,"offersCount":57,"supplierCount":25,"bestOffer":58},"785889752443260928","J-28559-9","\u002Fimages\u002Fchem_structures_svg\u002F292\u002F15060-55-6.svg","Ammonium tetrathiomolybdate","四硫钼酸铵","15060-55-6","H8MoN2S4","inorganic","无机化合物","Inorganic Chemicals",[23],7,{"unitPrice":59,"pack":28,"currency":29,"origin":30,"originFlag":31,"moq":21,"stock":32},24,{"slug":61,"cid":62,"imgSvg":63,"name":64,"nameZh":65,"cas":66,"formula":67,"iupac":64,"category":18,"categoryName":19,"categoryEnName":20,"industry":21,"tags":68,"offersCount":69,"supplierCount":25,"bestOffer":70},"855452191530422272","J-1994428-7","\u002Fimages\u002Fchem_structures_svg\u002F500\u002F16350-96-2.svg","N-benzylcyclohexanamine.hydrochloride","N-苄基环己胺盐酸盐","16350-96-2","C13H20ClN",[23],5,{"unitPrice":71,"pack":72,"currency":29,"origin":73,"originFlag":31,"moq":21,"stock":32},6,"1000g","中国",{"slug":75,"cid":76,"imgSvg":77,"name":78,"nameZh":79,"cas":80,"formula":81,"iupac":78,"category":18,"categoryName":19,"categoryEnName":20,"industry":21,"tags":82,"offersCount":25,"supplierCount":25,"bestOffer":83},"785871840001134592","J-982001-0","\u002Fimages\u002Fchem_structures_svg\u002F001\u002F10517-44-9.svg","1,3-Diaminopropane dihydrochloride","1,3-二氨基丙烷二盐酸盐","10517-44-9","C3H12Cl2N2",[23],{"unitPrice":84,"pack":28,"currency":29,"origin":30,"originFlag":31,"moq":21,"stock":32},3.78,{"slug":86,"cid":87,"imgSvg":88,"name":89,"nameZh":90,"cas":91,"formula":92,"iupac":89,"category":53,"categoryName":54,"categoryEnName":55,"industry":21,"tags":93,"offersCount":57,"supplierCount":94,"bestOffer":95},"785880365658017792","J-14975-6","\u002Fimages\u002Fchem_structures_svg\u002F262\u002F12003-67-7.svg","Lithium aluminate","氧化铝锂","12003-67-7","AlHLiO2",[23],3,{"unitPrice":94,"pack":28,"currency":29,"origin":73,"originFlag":31,"moq":21,"stock":32},{"slug":97,"cid":98,"imgSvg":99,"name":100,"nameZh":101,"cas":102,"formula":103,"iupac":100,"category":104,"categoryName":105,"categoryEnName":106,"industry":21,"tags":107,"offersCount":108,"supplierCount":108,"bestOffer":109},"785871830249377792","J-6931-9","\u002Fimages\u002Fchem_structures_svg\u002F456\u002F58068-80-7.svg","1-Octanoylbenzotriazole","N-辛酰苯并三唑","58068-80-7","C14H19N3O","aromatics","芳香烃","Aromatic Hydrocarbons",[],0,{"unitPrice":110,"pack":21,"currency":111,"origin":21,"originFlag":21,"moq":21,"stock":112},9007199254740991,"USD","made-to-order",{"slug":114,"cid":115,"imgSvg":116,"name":117,"nameZh":118,"cas":119,"formula":120,"iupac":117,"category":53,"categoryName":54,"categoryEnName":55,"industry":21,"tags":121,"offersCount":69,"supplierCount":25,"bestOffer":122},"785871842446413824","J-1960944-3","\u002Fimages\u002Fchem_structures_svg\u002F495\u002F10534-86-8.svg","Hexaamminecobalt(III) nitrate","六氨合钴(III)硝酸盐","10534-86-8","CoH18N9O9",[23],{"unitPrice":71,"pack":28,"currency":29,"origin":30,"originFlag":31,"moq":21,"stock":32},{"slug":124,"cid":125,"imgSvg":126,"name":127,"nameZh":128,"cas":129,"formula":130,"iupac":127,"category":53,"categoryName":54,"categoryEnName":55,"industry":21,"tags":131,"offersCount":24,"supplierCount":25,"bestOffer":132},"785871842576437248","J-814544-6","\u002Fimages\u002Fchem_structures_svg\u002F221\u002F12037-42-2.svg","vanadium oxide","氧化钒","12037-42-2","O13V6",[23],{"unitPrice":133,"pack":134,"currency":29,"origin":30,"originFlag":31,"moq":21,"stock":32},112.8,"100g",{"slug":136,"cid":137,"imgSvg":138,"name":139,"nameZh":140,"cas":141,"formula":142,"iupac":139,"category":53,"categoryName":54,"categoryEnName":55,"industry":21,"tags":143,"offersCount":57,"supplierCount":5,"bestOffer":144},"785871842647740416","J-1333345-2","\u002Fimages\u002Fchem_structures_svg\u002F479\u002F26490-63-1.svg","COBALT TETRAFLUOROBORATE HEXAHYDRATE","四氟化硼钴水合物","26490-63-1","B2CoF8H12O6",[23],{"unitPrice":145,"pack":134,"currency":29,"origin":30,"originFlag":31,"moq":21,"stock":32},12,{"slug":147,"cid":148,"imgSvg":149,"name":150,"nameZh":151,"cas":152,"formula":153,"iupac":150,"category":53,"categoryName":54,"categoryEnName":55,"industry":21,"tags":154,"offersCount":155,"supplierCount":94,"bestOffer":156},"785871842698072064","J-814953-0","\u002Fimages\u002Fchem_structures_svg\u002F432\u002F72259-10-0.svg","Copper(II) tetrafluoroborate hexahydrate","六水合四氟硼酸铜(II)","72259-10-0","B2CuF8H12O6",[23],10,{"unitPrice":157,"pack":28,"currency":29,"origin":21,"originFlag":21,"moq":21,"stock":32},2.6,{"slug":159,"cid":160,"imgSvg":161,"name":162,"nameZh":163,"cas":164,"formula":165,"iupac":162,"category":53,"categoryName":54,"categoryEnName":55,"industry":21,"tags":166,"offersCount":25,"supplierCount":5,"bestOffer":168},"785871863254355968","J-1990080-7","\u002Fimages\u002Fchem_structures_svg\u002F149\u002F123333-60-8.svg","Cerium(IV) sulfate hydrate","铈(IV)硫酸盐水合物","123333-60-8","CeH2O9S2",[167],"Made to Order",{"unitPrice":169,"pack":28,"currency":29,"origin":30,"originFlag":31,"moq":21,"stock":32},5.6,3204,{"slug":172,"cid":173,"imgSvg":174,"name":175,"nameZh":175,"cas":176,"formula":177,"iupac":175,"mdl":21,"mw":178,"category":179,"categoryName":180,"categoryEnName":181,"industry":21,"tags":182,"synonyms":183,"properties":188,"ghs":231,"offers":232,"activity":233,"upstream":252,"downstream":295,"customs":308,"safetyRows":309,"updatedAt":313,"isProhibited":314,"related":315,"__ts":351,"__lang":352,"__slug":173},"785895115771088896","J-1978433-5","\u002Fimages\u002Fchem_structures_svg\u002F062\u002F873054-44-5.svg","依伐卡托","873054-44-5","C24H28N2O3","392.491","organic","有机化合物","Organic Compounds",[],[184,185,186,187],"艾伐卡托","依法卡托","N-(2,4-二叔丁基-5-羟基苯基)-1,4-二氢-4-氧代-3-喹啉甲酰胺","依伐卡托杂质",[189,194,199,204,208,212,215,219,223,227],{"label":190,"labelZh":191,"value":192,"prop":193},"Boiling Point","沸点","550.5±50.0 °C at 760 mmHg","boilingPoint",{"label":195,"labelZh":196,"value":197,"prop":198},"Density","密度","1.2±0.1 g\u002Fcm3","density",{"label":200,"labelZh":201,"value":202,"prop":203},"Flash Point","闪点","286.7±30.1 °C","flashPoint",{"label":205,"labelZh":206,"value":207},"Exact Mass","精确质量","392.209991",{"label":209,"labelZh":210,"value":211},"PSA","其他信息","85.68000",{"label":213,"labelZh":213,"value":214},"LogP","6.34",{"label":216,"labelZh":217,"value":218},"Appearance","外观性状","白色 粉末",{"label":220,"labelZh":221,"value":222},"Vapor Pressure","蒸汽压","0.0±1.5 mmHg at 25°C",{"label":224,"labelZh":225,"value":226},"Refractive Index","折射率","1.606",{"label":228,"labelZh":229,"value":230},"Storage Conditions","储存条件","2~8℃",null,[],[234,237,240,243,246,249],{"key":235,"value":236},"描述","Ivacaftor是一种有效可口服的 CFTR 增效剂,靶向G551D-CFTR和F508del-CFTR的EC50分别为100 nM 和 25 nM。",{"key":238,"value":239},"相关类别","信号通路\n>>\n跨膜转运\n>>\nCFTR\n研究领域\n>>\n内分泌",{"key":241,"value":242},"靶点","EC50: 100 nM (G551D-CFTR), 25 nM (F508del-CFTR)[1]",{"key":244,"value":245},"体外研究","Ivacaftor(10μM)使ABCB4-G535D的PC分泌活性增加3倍,ABCB4-G536R增加13.7倍,ABCB4-S1076C增加6.7倍,ABCB4-S1176L增加9.4倍,ABCB4增加5.7倍-G1178S。 Ivacaftor纠正了ABCB4突变体的功能缺陷[1]。与R1162X CFTR细胞相比,Ivacaftor(10μM)显着增加表达W1282X的细胞中的CFTR活性[2]。 Ivacaftor显示对160个测试目标没有显着活性,包括GABAA苯二氮卓受体。 Ivacaftor增加氯离子分泌,EC50为0.236±0.200μM,与F508del HBE相比效力变化10倍[3]。在重组细胞中,VX-770增加F508del加工突变和G551D门控突变中的CFTR通道开放概率(Po)。 VX-770可将温度校正的F508del-FRT细胞中毛喉素刺激的IT增加6倍,EC50为25 nM [4]。",{"key":247,"value":248},"体内研究","Ivacaftor(1-200 mg\u002Fkg,po)在大鼠中表现出良好的口服生物利用度[3]。",{"key":250,"value":251},"参考文献","[1].\nDelaunay JL, et al. Functional defect of variants in the adenosine triphosphate-binding sites of ABCB4 and their rescue by the cystic fibrosis transmembrane conductance regulator potentiator, ivacaftor (VX-770). Hepatology. 2017 Feb;65(2):560-570\n[2].\nMutyam V, et al. Therapeutic benefit observed with the CFTR potentiator, ivacaftor, in a CF patient homozygous for the W1282X CFTR nonsense mutation. J Cyst Fibros. 2017 Jan;16(1):24-29\n[3].\nHadida S, et al. Discovery of N-(2,4-di-tert-butyl-5-hydroxyphenyl)-4-oxo-1,4-dihydroquinoline-3-carboxamide (VX-770, ivacaftor), a potent and orally bioavailable CFTR potentiator. J Med Chem. 2014 Dec 11;57(23):9776-9\n[4].\nVan Goor F, et al. Rescue of CF airway epithelial cell function in vitro by a CFTR potentiator, VX-770. Proc Natl Acad Sci U S A. 2009 Nov 3;106(44):18825-30.",[253,259,265,271,277,283,289],{"slug":254,"cid":255,"name":256,"nameZh":256,"cas":257,"imgSvg":258},"785888977642065900","J-1358148-0","5-氨基-2,4-二叔丁基苯酚","873055-58-4","https:\u002F\u002Fapi.chemical.xiaokangmed.com\u002Fimages\u002Fchem_structures_svg\u002F347\u002F873055-58-4.svg",{"slug":260,"cid":261,"name":262,"nameZh":262,"cas":263,"imgSvg":264},"785895141138239500","J-1507529-9","5-氨基-2,4-二叔丁基苯甲酸甲酯","1182822-31-6","https:\u002F\u002Fapi.chemical.xiaokangmed.com\u002Fimages\u002Fchem_structures_svg\u002F058\u002F1182822-31-6.svg",{"slug":266,"cid":267,"name":268,"nameZh":268,"cas":269,"imgSvg":270},"785896710969757700","J-1516776-3","4-氧代-1,4-二氢喹啉-3-羧酸","13721-01-2","https:\u002F\u002Fapi.chemical.xiaokangmed.com\u002Fimages\u002Fchem_structures_svg\u002F299\u002F13721-01-2.svg",{"slug":272,"cid":273,"name":274,"nameZh":274,"cas":275,"imgSvg":276},"785871833726455800","J-52594-5","2,4-二叔丁基酚","96-76-4","https:\u002F\u002Fapi.chemical.xiaokangmed.com\u002Fimages\u002Fchem_structures_svg\u002F210\u002F96-76-4.svg",{"slug":278,"cid":279,"name":280,"nameZh":280,"cas":281,"imgSvg":282},"785894849810272300","J-1505249-6","2,4-二叔丁基-5-硝基苯酚甲酸甲酯","873055-55-1","https:\u002F\u002Fapi.chemical.xiaokangmed.com\u002Fimages\u002Fchem_structures_svg\u002F461\u002F873055-55-1.svg",{"slug":284,"cid":285,"name":286,"nameZh":286,"cas":287,"imgSvg":288},"785876783596572700","J-1099502-6","1,4-二氢-4-氧代-3-喹啉羧酸乙酯","52980-28-6","https:\u002F\u002Fapi.chemical.xiaokangmed.com\u002Fimages\u002Fchem_structures_svg\u002F272\u002F52980-28-6.svg",{"slug":290,"cid":291,"name":292,"nameZh":292,"cas":293,"imgSvg":294},"785898150379393000","J-1452837-0","2-苯基氨基亚甲基-丙二酸二乙酯","54535-22-7","https:\u002F\u002Fapi.chemical.xiaokangmed.com\u002Fimages\u002Fchem_structures_svg\u002F275\u002F54535-22-7.svg",[296,302],{"slug":297,"cid":298,"name":299,"nameZh":299,"cas":300,"imgSvg":301},"785892176688058400","J-1973215-8","N-(2-(叔丁基)-5-羟基...","1246213-23-9","https:\u002F\u002Fapi.chemical.xiaokangmed.com\u002Fimages\u002Fchem_structures_svg\u002F441\u002F1246213-23-9.svg",{"slug":303,"cid":304,"name":305,"nameZh":305,"cas":306,"imgSvg":307},"785892176730001400","J-1819609-4","4-[[(1,4-二氢-4-氧...","1246213-24-0","https:\u002F\u002Fapi.chemical.xiaokangmed.com\u002Fimages\u002Fchem_structures_svg\u002F393\u002F1246213-24-0.svg",[],[310],{"key":311,"value":312},"海关编码","29333990","2026-08-21T07:51:12.859463Z",false,[316,322,328,333,339,345],{"slug":317,"cid":318,"name":319,"nameZh":320,"cas":321},"785882572553981952","J-1110759-4","Methyl Violet 2B","甲基紫2B","8004-87-3",{"slug":323,"cid":324,"name":325,"nameZh":326,"cas":327},"785884696738271232","J-1324442-0","Ethylenediaminetetraacetate-copper-ammonia complex","EDTA-铜铵络合物","67989-88-2",{"slug":329,"cid":330,"name":331,"nameZh":331,"cas":332},"785911446427930624","J-69315-4","9-Bromo-10-(naphthalen-1-yl-d7)anthracene-1,2,3,4,5,6,7,8-d8","2330770-73-3",{"slug":334,"cid":335,"name":336,"nameZh":337,"cas":338},"785898743068102656","J-1365189-3","Esomeprazole calcium","埃索美拉唑钙","161796-85-6",{"slug":340,"cid":341,"name":342,"nameZh":343,"cas":344},"785884051318771712","J-1114131-2","Alizarin Complexone","茜素络合指示剂","3952-78-1",{"slug":346,"cid":347,"name":348,"nameZh":349,"cas":350},"785896769497075712","J-45303-5","Cholecalciferol","维生素D3","67-97-0",1792671999068,"zh-CN",[]]