Lumacaftor (VX-809)

For research use only. Not for use in humans.

目录号:S1565 别名: VRT 826809

Lumacaftor (VX-809) Chemical Structure

CAS No. 936727-05-8

Lumacaftor (VX-809, VRT 826809)通过促进突变型CFTR(F508del-CFTR)的成熟,从而纠正囊性纤维症中常见的CFTR突变,在fisher大鼠甲状腺细胞中EC50为0.1 μM。Phase 3。

规格 价格 库存 购买数量  
10mM (1mL in DMSO) RMB 2775.91 现货
RMB 1412.12 现货
RMB 2626.25 现货
RMB 7923.73 现货
RMB 16298.1 现货
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客户使用Selleck生产的Lumacaftor (VX-809)发表文献164篇:

产品安全说明书

CFTR抑制剂选择性比较

生物活性

产品描述 Lumacaftor (VX-809, VRT 826809)通过促进突变型CFTR(F508del-CFTR)的成熟,从而纠正囊性纤维症中常见的CFTR突变,在fisher大鼠甲状腺细胞中EC50为0.1 μM。Phase 3。
特性 在纠正CFTR缺陷方面,VX-809比之前报道的药物特异性更强,且更有效。
靶点
F508del-CFTR [1]
(Fisher rat thyroid cells)
0.1 μM(EC50)
体外研究

VX-809在雌激素受体(ER)水平上起作用的行为,使一部分F508del-CFTR采取正确折叠的方式,退出ER,转移到细胞表面,正常起作用。VX-809作用于表达 F508del-CFTR的Fischer 大鼠甲状腺 (FRT)细胞, VX-809显著提高F508del-CFTR突变,提高7.1 倍,EC50为0.1 μM, 且增强F508del-CFTR调节的氯离子运输,提高5 倍, EC50 为0.5 μM, 而VRT-768作用时具有更高的EC50 值,EC50分别为7.9 μM 和 16 μM。VX-809 (3 μM)作用于表达F508del-CFTR的HEK-293细胞,提高ER中的F508del-CFTR,提高6倍。VX-809作用于携带F508del-CFTR突变的原代人支气管上皮 (HBE)细胞,提高CFTR成熟度,且增强氯离子分泌,EC50分别为350 nM和81 nM,比Corr-4a和VRT-325更有效。VX-809修正的F508del-CFTR具有单通道开发概率,为0.39,与正常CFTR 类似,正常CFTR为0.40。与VX-770不同, VX-809不是CFTR增强剂,急剧加入VX-809不会对F508del-CFTR 功能造成影响。与VRT-325和Corr-4a相反, VX-809 不会促进正常或突变形式hERG 或P-gp的进程,及其他疾病引起错误定位的蛋白质,包括α1-抗胰蛋白酶Z突变 (E342K-α1-AT)或N370S-β-葡萄糖苷酶, 说明VX-809特异性作用于CFTR。

Cell Data
Cell Lines Assay Type Concentration Incubation Time Formulation Activity Description PMID
CFBE41o NVvSVIIyS2:{cnXjeI9zKGG|c3H5 NUj4bJB[OjRiaILz MYrDc5Jz\WO2b4KgZYN1cX[rdImgZZQhS0[WUjDGOVA5NWSnbDDteZRidnRiKIXub45wf25ib4Lp[4lvMSCneIDy[ZN{\WRiaX6gbJVu[W5iQ1\CSVQydyClZXzsd{Bp[XKkb4LpcochUFNvWV\QJJBz\WmwY4XiZZRm\CCob4KgNlQhcHK|IH\vcIxwf2WmIHL5JIZwenOtb3zpck9o\W6rc4TlbY4he3SrbYXsZZRqd25iZn;yJFMxKG2rboOgZpkh\my3b4Lld4NmdmOnIHHzd4F6NCCHQ{WwJF0hOi53N{C0JO69VS5? M2r4NlxiKHSjcnfleF0oZ2KuYX7rK{BpemWoPTfoeJRxezpxL4D1Zo1m\C6wY3LpMo5tdS6waXiu[493NzJ7MkeyO|Q6Lz5{OUK3Nlc1QTxxYU6=
FRT Mlr4R49zemWldH;yJIF{e2G7 NH[0RWEzPSCvaX7z NXzLW4lbS2:{cnXjeI9zKGGldHn2bZR6KGG2IHj1cYFvKEOIVGKgSlUxQCCmZXzleIlwdiCvdYThcpQh\XiycnXzd4VlKGmwIF\SWEBk\WyuczDpcoN2[mG2ZXSg[o9zKDJ3IH3pcpMhf2m2aDDmc5J{c2:uaX6gZpkhYU[SLXLhd4VlKG[udX;y[ZNk\W6lZTDhcoFtgXOrczDy[YxifGm4ZTD0c{Bkd262cn;sMEBGSzVyIE2gNk43KM7:TT6= NIPYdlY9[SC2YYLn[ZQ:L1:kbHHub{chcHKnZk2nbJR1eHN8Lz;weYJu\WRwbnPibU5vdG1wbnnoModwfi9{NkW2NVAxOyd-Mk[1OlExODN:L3G+
TC32 NUTDZ2JPeUiWUzDhd5NigQ>? MVLxTHRUKG:oIIDl[IlifHKrYzDjZY5k\XJiY3XscEBtcW6nczD0c{Bq\GWwdHnmfUBufWy2aYDs[UBweHCxcoT1col1cWW|IH\vdkBlenWpIILldJVzeG:|aX7nPkBRemmvYYL5JJNkemWnbjDmc5IhXEN|MjDj[Yxtew>? MXS8ZUB1[XKpZYS9K39jdGGwazegbJJm\j1paIT0dJM7Ny:ydXLt[YQvdmOkaT7ucI0vdmmqLnfvek8zQTR|NUGzPUc,Ojl2M{WxN|k9N2F-
U-2 OS M2rXe5FJXFNiYYPzZZk> MlL4dWhVWyCxZjDw[YRq[XS{aXOgZ4Fv[2W{IHPlcIwhdGmwZYOgeI8hcWSnboTp[pkhdXWudHnwcIUhd3Cyb4L0eY5qfGmnczDmc5Ih\HK3ZzDy[ZB2enCxc3nu[|ohWHKrbXHyfUB{[3KnZX6g[o9zKFVvMjDPV{Bk\Wyucx?= NFH0eo89[SC2YYLn[ZQ:L1:kbHHub{chcHKnZk2nbJR1eHN8Lz;weYJu\WRwbnPibU5vdG1wbnnoModwfi9{OUSzOVE{QSd-Mkm0N|UyOzl:L3G+
A673 MorMdWhVWyCjc4PhfS=> MVjxTHRUKG:oIIDl[IlifHKrYzDjZY5k\XJiY3XscEBtcW6nczD0c{Bq\GWwdHnmfUBufWy2aYDs[UBweHCxcoT1col1cWW|IH\vdkBlenWpIILldJVzeG:|aX7nPkBRemmvYYL5JJNkemWnbjDmc5IhSTZ5MzDj[Yxtew>? NWrsd3NORGFidHHy[4V1RSehYnzhcosoKGi{ZX[9K4h1fHC|Oj:vdJVjdWWmLn7jZokvdmyvLn7pbE5od3ZxMkm0N|UyOzlpPkK5OFM2OTN7PD;hQi=>
DAOY NXzRfW9OeUiWUzDhd5NigQ>? M4TkXJFJXFNib3[gdIVlcWG2cnnjJINidmOncjDj[YxtKGyrbnXzJJRwKGmmZX70bYZ6KG23bITpdIxmKG:ycH;yeJVvcXSrZYOg[o9zKGS{dXegdoVxfXKyb4Ppcoc7KFC{aX3hdpkhe2O{ZXXuJIZweiCGQV;ZJINmdGy| NXu1bWZPRGFidHHy[4V1RSehYnzhcosoKGi{ZX[9K4h1fHC|Oj:vdJVjdWWmLn7jZokvdmyvLn7pbE5od3ZxMkm0N|UyOzlpPkK5OFM2OTN7PD;hQi=>
Saos-2 MXnxTHRUKGG|c3H5 MmXUdWhVWyCxZjDw[YRq[XS{aXOgZ4Fv[2W{IHPlcIwhdGmwZYOgeI8hcWSnboTp[pkhdXWudHnwcIUhd3Cyb4L0eY5qfGmnczDmc5Ih\HK3ZzDy[ZB2enCxc3nu[|ohWHKrbXHyfUB{[3KnZX6g[o9zKFOjb4OtNkBk\Wyucx?= NYn5W2F2RGFidHHy[4V1RSehYnzhcosoKGi{ZX[9K4h1fHC|Oj:vdJVjdWWmLn7jZokvdmyvLn7pbE5od3ZxMkm0N|UyOzlpPkK5OFM2OTN7PD;hQi=>
BT-37 NVPlVHc3eUiWUzDhd5NigQ>? M{OxfJFJXFNib3[gdIVlcWG2cnnjJINidmOncjDj[YxtKGyrbnXzJJRwKGmmZX70bYZ6KG23bITpdIxmKG:ycH;yeJVvcXSrZYOg[o9zKGS{dXegdoVxfXKyb4Ppcoc7KFC{aX3hdpkhe2O{ZXXuJIZweiCEVD2zO{Bk\Wyucx?= NGCw[HY9[SC2YYLn[ZQ:L1:kbHHub{chcHKnZk2nbJR1eHN8Lz;weYJu\WRwbnPibU5vdG1wbnnoModwfi9{OUSzOVE{QSd-Mkm0N|UyOzl:L3G+
RD M{faeJFJXFNiYYPzZZk> M4fpWpFJXFNib3[gdIVlcWG2cnnjJINidmOncjDj[YxtKGyrbnXzJJRwKGmmZX70bYZ6KG23bITpdIxmKG:ycH;yeJVvcXSrZYOg[o9zKGS{dXegdoVxfXKyb4Ppcoc7KFC{aX3hdpkhe2O{ZXXuJIZweiCURDDj[Yxtew>? Mn63QIEhfGG{Z3X0QUdg[myjbnunJIhz\WZ;J3j0eJB{Qi9xcIXicYVlNm6lYnmucoxuNm6raD7nc5YwOjl2M{WxN|koRjJ7NEO1NVM6RC:jPh?=
SK-N-SH MlvZdWhVWyCjc4PhfS=> MVLxTHRUKG:oIIDl[IlifHKrYzDjZY5k\XJiY3XscEBtcW6nczD0c{Bq\GWwdHnmfUBufWy2aYDs[UBweHCxcoT1col1cWW|IH\vdkBlenWpIILldJVzeG:|aX7nPkBRemmvYYL5JJNkemWnbjDmc5IhW0tvTj3TTEBk\Wyucx?= NEPyN|c9[SC2YYLn[ZQ:L1:kbHHub{chcHKnZk2nbJR1eHN8Lz;weYJu\WRwbnPibU5vdG1wbnnoModwfi9{OUSzOVE{QSd-Mkm0N|UyOzl:L3G+
BT-12 MmjOdWhVWyCjc4PhfS=> MnvEdWhVWyCxZjDw[YRq[XS{aXOgZ4Fv[2W{IHPlcIwhdGmwZYOgeI8hcWSnboTp[pkhdXWudHnwcIUhd3Cyb4L0eY5qfGmnczDmc5Ih\HK3ZzDy[ZB2enCxc3nu[|ohWHKrbXHyfUB{[3KnZX6g[o9zKEKWLUGyJINmdGy| M33UclxiKHSjcnfleF0oZ2KuYX7rK{BpemWoPTfoeJRxezpxL4D1Zo1m\C6wY3LpMo5tdS6waXiu[493NzJ7NEO1NVM6Lz5{OUSzOVE{QTxxYU6=
MG 63 (6-TG R) NF2wSGlyUFSVIHHzd4F6 M3TlR5FJXFNib3[gdIVlcWG2cnnjJINidmOncjDj[YxtKGyrbnXzJJRwKGmmZX70bYZ6KG23bITpdIxmKG:ycH;yeJVvcXSrZYOg[o9zKGS{dXegdoVxfXKyb4Ppcoc7KFC{aX3hdpkhe2O{ZXXuJIZweiCPRzC2N{ApPi2WRzDSLUBk\Wyucx?= NFzOUIc9[SC2YYLn[ZQ:L1:kbHHub{chcHKnZk2nbJR1eHN8Lz;weYJu\WRwbnPibU5vdG1wbnnoModwfi9{OUSzOVE{QSd-Mkm0N|UyOzl:L3G+
NB1643 M{PQUJFJXFNiYYPzZZk> M{fzfpFJXFNib3[gdIVlcWG2cnnjJINidmOncjDj[YxtKGyrbnXzJJRwKGmmZX70bYZ6KG23bITpdIxmKG:ycH;yeJVvcXSrZYOg[o9zKGS{dXegdoVxfXKyb4Ppcoc7KFC{aX3hdpkhe2O{ZXXuJIZweiCQQkG2OFMh[2WubIO= NILFcnI9[SC2YYLn[ZQ:L1:kbHHub{chcHKnZk2nbJR1eHN8Lz;weYJu\WRwbnPibU5vdG1wbnnoModwfi9{OUSzOVE{QSd-Mkm0N|UyOzl:L3G+
BT-12 MYPxTHRUKGG|c3H5 M3LMUpFJXFNib3[gdIVlcWG2cnnjJINidmOncjDj[YxtKGyrbnXzJJRwKGmmZX70bYZ6KG23bITpdIxmKG:ycH;yeJVvcXSrZYOg[o9zKGS{dXegdoVxfXKyb4Ppcoc7KEOxbn\pdo1ifG:{eTDzZ5Jm\W5iZn;yJGJVNTF{IHPlcIx{ M3vtTlxiKHSjcnfleF0oZ2KuYX7rK{BpemWoPTfoeJRxezpxL4D1Zo1m\C6wY3LpMo5tdS6waXiu[493NzJ7NEO1NVM6Lz5{OUSzOVE{QTxxYU6=
OHS-50 M4PJWpFJXFNiYYPzZZk> M4LnWpFJXFNib3[gdIVlcWG2cnnjJINidmOncjDj[YxtKGyrbnXzJJRwKGmmZX70bYZ6KG23bITpdIxmKG:ycH;yeJVvcXSrZYOg[o9zKGS{dXegdoVxfXKyb4Ppcoc7KFC{aX3hdpkhe2O{ZXXuJIZweiCRSGOtOVAh[2WubIO= MkPwQIEhfGG{Z3X0QUdg[myjbnunJIhz\WZ;J3j0eJB{Qi9xcIXicYVlNm6lYnmucoxuNm6raD7nc5YwOjl2M{WxN|koRjJ7NEO1NVM6RC:jPh?=
fibroblast cells MlrXdWhVWyCjc4PhfS=> M2jyVJFJXFNib3[gdIVlcWG2cnnjJINidmOncjDj[YxtKGyrbnXzJJRwKGmmZX70bYZ6KG23bITpdIxmKG:ycH;yeJVvcXSrZYOg[o9zKGS{dXegdoVxfXKyb4Ppcoc7KFC{aX3hdpkhe2O{ZXXuJIZweiClb370do9tKEiqIIfpcIQhfHmyZTDmbYJzd2KuYYP0JINmdGy| NV7oem05RGFidHHy[4V1RSehYnzhcosoKGi{ZX[9K4h1fHC|Oj:vdJVjdWWmLn7jZokvdmyvLn7pbE5od3ZxMkm0N|UyOzlpPkK5OFM2OTN7PD;hQi=>
Rh41 MXXxTHRUKGG|c3H5 M4nlfJFJXFNib3[gdIVlcWG2cnnjJINidmOncjDj[YxtKGyrbnXzJJRwKGmmZX70bYZ6KG23bITpdIxmKG:ycH;yeJVvcXSrZYOg[o9zKGS{dXegdoVxfXKyb4Ppcoc7KFC{aX3hdpkhe2O{ZXXuJIZweiCUaESxJINmdGy| MmT2QIEhfGG{Z3X0QUdg[myjbnunJIhz\WZ;J3j0eJB{Qi9xcIXicYVlNm6lYnmucoxuNm6raD7nc5YwOjl2M{WxN|koRjJ7NEO1NVM6RC:jPh?=
Rh30 MXfxTHRUKGG|c3H5 MWLxTHRUKG:oIIDl[IlifHKrYzDjZY5k\XJiY3XscEBtcW6nczD0c{Bq\GWwdHnmfUBufWy2aYDs[UBweHCxcoT1col1cWW|IH\vdkBlenWpIILldJVzeG:|aX7nPkBEd26oaYLtZZRwenlic3Py[YVvKG[xcjDSbFMxKGOnbHzz NIHmRXg9[SC2YYLn[ZQ:L1:kbHHub{chcHKnZk2nbJR1eHN8Lz;weYJu\WRwbnPibU5vdG1wbnnoModwfi9{OUSzOVE{QSd-Mkm0N|UyOzl:L3G+
U-2 OS NHK3THdyUFSVIHHzd4F6 M{\PZpFJXFNib3[gdIVlcWG2cnnjJINidmOncjDj[YxtKGyrbnXzJJRwKGmmZX70bYZ6KG23bITpdIxmKG:ycH;yeJVvcXSrZYOg[o9zKGS{dXegdoVxfXKyb4Ppcoc7KEOxbn\pdo1ifG:{eTDzZ5Jm\W5iZn;yJHUuOiCRUzDj[Yxtew>? M1TtfVxiKHSjcnfleF0oZ2KuYX7rK{BpemWoPTfoeJRxezpxL4D1Zo1m\C6wY3LpMo5tdS6waXiu[493NzJ7NEO1NVM6Lz5{OUSzOVE{QTxxYU6=
Rh41 MkCzdWhVWyCjc4PhfS=> NGjSN2VyUFSVIH;mJJBm\GmjdILpZ{Bk[W6lZYKgZ4VtdCCuaX7ld{B1dyCrZHXueIlngSCvdXz0bZBt\SCxcIDvdpR2dmm2aXXzJIZweiCmcoXnJJJmeHW{cH;zbY5oQiCFb37mbZJu[XSxcomgd4Nz\WWwIH\vdkBTcDRzIHPlcIx{ Mo\mQIEhfGG{Z3X0QUdg[myjbnunJIhz\WZ;J3j0eJB{Qi9xcIXicYVlNm6lYnmucoxuNm6raD7nc5YwOjl2M{WxN|koRjJ7NEO1NVM6RC:jPh?=
RD NHvjWXNyUFSVIHHzd4F6 MlHBdWhVWyCxZjDw[YRq[XS{aXOgZ4Fv[2W{IHPlcIwhdGmwZYOgeI8hcWSnboTp[pkhdXWudHnwcIUhd3Cyb4L0eY5qfGmnczDmc5Ih\HK3ZzDy[ZB2enCxc3nu[|ohS2:wZnnycYF1d3K7IIPjdoVmdiCob4KgVmQh[2WubIO= M1TyN|xiKHSjcnfleF0oZ2KuYX7rK{BpemWoPTfoeJRxezpxL4D1Zo1m\C6wY3LpMo5tdS6waXiu[493NzJ7NEO1NVM6Lz5{OUSzOVE{QTxxYU6=
SJ-GBM2 NGW3bmZyUFSVIHHzd4F6 NXT2OWo4eUiWUzDv[kBx\WSrYYTybYMh[2GwY3XyJINmdGxibHnu[ZMhfG9iaXTlcpRq\nlibYXseIlxdGVib4Dwc5J1fW6rdHnld{Bnd3JiZIL1[{Bz\XC3coDvd4lv\zpiUILpcYFzgSC|Y4Ll[Y4h\m:{IGPKMWdDVTJiY3XscJM> MmjOQIEhfGG{Z3X0QUdg[myjbnunJIhz\WZ;J3j0eJB{Qi9xcIXicYVlNm6lYnmucoxuNm6raD7nc5YwOjl2M{WxN|koRjJ7NEO1NVM6RC:jPh?=
SK-N-MC MU\xTHRUKGG|c3H5 M4ezcJFJXFNib3[gdIVlcWG2cnnjJINidmOncjDj[YxtKGyrbnXzJJRwKGmmZX70bYZ6KG23bITpdIxmKG:ycH;yeJVvcXSrZYOg[o9zKGS{dXegdoVxfXKyb4Ppcoc7KFC{aX3hdpkhe2O{ZXXuJIZweiCVSz3OMW1EKGOnbHzz MlKzQIEhfGG{Z3X0QUdg[myjbnunJIhz\WZ;J3j0eJB{Qi9xcIXicYVlNm6lYnmucoxuNm6raD7nc5YwOjl2M{WxN|koRjJ7NEO1NVM6RC:jPh?=
NB-EBc1 MXvxTHRUKGG|c3H5 MWHxTHRUKG:oIIDl[IlifHKrYzDjZY5k\XJiY3XscEBtcW6nczD0c{Bq\GWwdHnmfUBufWy2aYDs[UBweHCxcoT1col1cWW|IH\vdkBlenWpIILldJVzeG:|aX7nPkBRemmvYYL5JJNkemWnbjDmc5IhVkJvRVLjNUBk\Wyucx?= MnraQIEhfGG{Z3X0QUdg[myjbnunJIhz\WZ;J3j0eJB{Qi9xcIXicYVlNm6lYnmucoxuNm6raD7nc5YwOjl2M{WxN|koRjJ7NEO1NVM6RC:jPh?=
LAN-5 NX3R[YF{eUiWUzDhd5NigQ>? MnrGdWhVWyCxZjDw[YRq[XS{aXOgZ4Fv[2W{IHPlcIwhdGmwZYOgeI8hcWSnboTp[pkhdXWudHnwcIUhd3Cyb4L0eY5qfGmnczDmc5Ih\HK3ZzDy[ZB2enCxc3nu[|ohWHKrbXHyfUB{[3KnZX6g[o9zKEyDTj21JINmdGy| NH7LWlU9[SC2YYLn[ZQ:L1:kbHHub{chcHKnZk2nbJR1eHN8Lz;weYJu\WRwbnPibU5vdG1wbnnoModwfi9{OUSzOVE{QSd-Mkm0N|UyOzl:L3G+
Rh18 NWrrU|JLeUiWUzDhd5NigQ>? NHT1RZlyUFSVIH;mJJBm\GmjdILpZ{Bk[W6lZYKgZ4VtdCCuaX7ld{B1dyCrZHXueIlngSCvdXz0bZBt\SCxcIDvdpR2dmm2aXXzJIZweiCmcoXnJJJmeHW{cH;zbY5oQiCScnntZZJ6KHOlcnXlckBnd3JiUnixPEBk\Wyucx?= M1O2NFxiKHSjcnfleF0oZ2KuYX7rK{BpemWoPTfoeJRxezpxL4D1Zo1m\C6wY3LpMo5tdS6waXiu[493NzJ7NEO1NVM6Lz5{OUSzOVE{QTxxYU6=
CFBE41o NH6zV|FEd3K{ZXP0c5Ih[XO|YYm= M33OSFEhfU1? MYOyOEBpenN? NFvKOWNEd3K{ZXP0c5Ih[WO2aY\peJkh[XRiQ1\UVkBHPTB6LXTlcEBufXSjboSgLJVvc26xd36gc5Jq\2mwKTDlfJBz\XO|ZXSgbY4hcHWvYX6gR2ZDTTRzbzDj[YxteyCqYYLic5JqdmdiSGOtXWZRKGG|c3Xzd4VlKGG|IHnuZ5Jm[XOnIHnuJI1ifHW{ZXSgdJJwfGWrbjDs[ZZmdHNiYYSgZ4VtdCC|dYLmZYNmKGG2IEGgeW0h[W[2ZYKgNlQhcHK|IHL5JIVt\WO2cn;wbI9z\XSrYzDtc4JqdGm2eTDhd5NigQ>? MWK8ZUB1[XKpZYS9K39jdGGwazegbJJm\j1paIT0dJM7Ny:ydXLt[YQvdmOkaT7ucI0vdmmqLnfvek8zQTJ5Mke0PUc,Ojl{N{K3OFk9N2F-
HBE MnjpR49zemWldH;yJIF{e2G7 NUPK[oM3S2:{cnXjeI9zKGGldHn2bZR6KGG2IFPGWHIhTjVyOHTlcE9HPTB6ZHXsJI12fGGwdDDpckBxemmvYYL5JGhDTSClZXzsd{Bie3Onc4Pl[EBieyCrbnPy[YF{\SCrbjDjbIxwemmmZTDpc44h[3W{cnXueEBi[3Kxc4OgZZBq[2GuIH3lcYJz[W6nIH3lZZN2emWmIEG4JJRwKDJ2IHjyd{Bxd3O2IHPvcZBwfW6mIITy[YF1dWWwdDDvckBj[XOxbHH0[ZJidCC|aXTlJI9nKGOnbHzzJIlvKHC{ZYPlcoNmKG:oIHPoZY5v\WxicH;0[Y51cWG2b4KgS2xRTzF6 NIDK[GY9[SC2YYLn[ZQ:L1:kbHHub{chcHKnZk2nbJR1eHN8Lz;weYJu\WRwbnPibU5vdG1wbnnoModwfi9{OUK1NVk{Oid-MkmyOVE6OzJ:L3G+
CFBE41o M1r6PGNwenKnY4TvdkBie3OjeR?= MWDDc5Jz\WO2b4KgZYN1cX[rdImgZZQhS0[WUjDGOVA5\GWuIH31eIFvfCBqdX7rco94diCxcnnnbY4qKGW6cILld5Nm\CCrbjDoeY1idiCFRlLFOFFwKGOnbHzzJIF{e2W|c3XkJIF{KGmwY4LlZZNmKGmwIH\1cIx6KGeueXPvd5lt[XSnZDDwdo91\WmwIHL5JJdme3Sncn6gZoxwfCCjbnHsfZNqew>? NHPKZ5A9[SC2YYLn[ZQ:L1:kbHHub{chcHKnZk2nbJR1eHN8Lz;weYJu\WRwbnPibU5vdG1wbnnoModwfi9{NkC0NVU4Pyd-Mk[wOFE2Pzd:L3G+
CFBE41o M4HqemNwenKnY4TvdkBie3OjeR?= MUWxJJVO NWr3c4R{OjRiaILz NFe1XmhEd3K{ZXP0c5Ih[WO2aY\peJkh[XRiQ1\UVkBHPTB6ZHXsJI12fGGwdDCoeY5sdm:5bjDvdolocW5rIHX4dJJme3OnZDDpckBpfW2jbjDDSmJGPDGxIHPlcIx{KGG|c3Xzd4VlKGG|IHnuZ5Jm[XOnIHnuJJNqgmVib3[gZ2FOWC2mZYDlcoRmdnRiY4XydoVvfCCjdDCxJJVOKGGodHXyJFI1KGi{czDt[YF{fXKnZDDheEAsOTByIH3WJIJ6KHeqb3zlJINmdGxicHH0Z4gh[2yjbYCgZZN{[Xl? NVP5NJhpRGFidHHy[4V1RSehYnzhcosoKGi{ZX[9K4h1fHC|Oj:vdJVjdWWmLn7jZokvdmyvLn7pbE5od3ZxMk[wOFE2PzdpPkK2NFQyPTd5PD;hQi=>

... Click to View More Cell Line Experimental Data

Assay
Methods Test Index PMID
Immunofluorescence
CFTR / USP13; 

PubMed: 30618756     


Confocal microscope images taken from CFBE41o- cells stained with antibodies against CFTR and USP13. Cells were treated with VX-809 (1 μM) or vehicle. Arrows indicate examples of cells with peripheral co-localization of F508del-CFTR and USP13. Arrowheads show peripheral regions with USP13 alone. Insets depict magnified cell areas of cell periphery with colocalization of USP13 and F508del-CFTR.

Cell surface kAE1 / kAE1; 

PubMed: 23460825     


MDCK cells expressing WT or kAE1 mutant were either kept in control conditions or treated with 3 µM of the molecular chaperone VX-809 for 24 hours prior to fixation. We then performed a two-step immunostaining as follows: cells were blocked and stained with a mouse anti-HA antibody followed by staining with an Alexa 488 coupled secondary antibody. Cells were then permeabilized and after blocking, stained again with a mouse anti-HA antibody followed by a Cy3 coupled secondary antibody. The samples were examined with a WaveFx spinning disk using a 60×oil immersion objective. The size bar corresponds to 10 µm

30618756 23460825

推荐的实验操作(此推荐来自于公开的文献所以Selleck并不保证其有效性)

激酶实验:[1]
- 合并

F508del-CFTR成熟度:

用浓度不断增高的VX-809处理稳定表达F508del-CFTR的Fischer大鼠甲状腺(FRT)细胞48小时。温育后,细胞收集在冰冻D-PBS溶液中 (没有钙和镁),然后按1,000 × g 转速在4oC下离心。细胞颗粒溶解1% Nonidet P-40, 0.5%去氧胆酸钠,200 mM NaCl, 10 mM Tris, pH 7.8, 及 1 mM EDTA和蛋白酶抑制剂混合物中(1:250),然后在冰上放置30分钟。裂解物在10,000×g转速4oC下旋转10分钟,将细胞核和不溶性物质制成颗粒状。12 μg 全部蛋白在Laemmli buffer 中与5% β-巯基乙醇在37oC加热5分钟,然后上样到3% 到8% Tris-乙酸凝胶上。凝胶转移到硝化纤维素中,使用单克隆CFTR抗体或 GAPDH多克隆抗体进行 Western blotting。通过增强发光而进行印迹。通过扫描片的 NIH ImageJ 分析而而测量C带和GAPDH的相对量。
细胞实验:[1]
- 合并
  • Cell lines: FRT(CFTR 或 F508del-CFTR), HEK-293 (CFTT 或 F508del-CFTR), 和HBE
  • Concentrations: 溶于DMSO, 终浓度为~0.1 mM
  • Incubation Time: 24 或 48 小时
  • Method: 使用浓度不断增高的VX-809处理细胞24或48小时。使用Ussing chamber技术记录 CFTR介导氯离子运输中的跨膜电流 (IT)结果。通过使用离体的内面外向式膜片钳测量CFTR单通道活性。使用单克隆CFTR 抗体及免疫印迹技术测量表达CFTR或F508del-CFTR的FRT, HEK-293, 或 HBE细胞中CFTR成熟度。
    (Only for Reference)

溶解度 (25°C)

体外 DMSO 90 mg/mL (198.93 mM)
Water Insoluble
Ethanol '6 mg/mL
体内 从左到右依次将纯溶剂加入产品,现配现用(数据来自Selleck实验检测而非文献):
30% PEG400+0.5% Tween80+5% propylene glycol
30 mg/mL

* 溶解度检测是由Selleck技术部门检测的,可能会和文献中提供的溶解度有所差异,这是由于生产工艺和批次不同产生的正常现象。请按照顺序依次加入各个纯溶剂。

化学数据

分子量 452.41
化学式

C24H18F2N2O5

CAS号 936727-05-8
储存条件 粉状
溶于溶剂
别名 VRT 826809

动物体内配方计算器 (澄清溶液)

第一步:请输入基本实验信息(考虑到实验过程中的损耗,建议多配一只动物的药量)
给药剂量 mg/kg 动物平均体重 g 每只动物给药体积 ul 动物数量
第二步:请输入动物体内配方组成(配方适用于不溶于水的药物;不同批次药物配方比例不同,请联系Selleck为您提供正确的澄清溶液配方)
% DMSO % % Tween 80 % ddH2O
计算重置

计算器

摩尔浓度计算器

摩尔浓度计算器

本计算器可帮助您计算出特定溶液中溶质的质量、溶液浓度和体积之间的关系,公式为:

质量 (mg) = 浓度 (mM) x 体积 (mL) x 分子量 (g/mol)

摩尔浓度计算公式

  • 质量
    浓度
    体积
    分子量

*在配置溶液时,请务必参考Selleck产品标签上、MSDS / COA(可在Selleck的产品页面获得)批次特异的分子量使用本工具。

稀释计算器

稀释计算器

用本工具协助配置特定浓度的溶液,使用的计算公式为:

开始浓度 x 开始体积 = 最终浓度 x 最终体积

稀释公式

稀释公式一般简略地表示为: C1V1 = C2V2 ( 输入 输出 )

  • C1
    V1
    C2
    V2

在配置溶液时,请务必参考Selleck产品标签上、MSDS / COA(可在Selleck的产品页面获得)批次特异的分子量使用本工具。.

连续稀释计算器方程

  • 连续稀释

  • 计算结果

  • C1=C0/X C1: LOG(C1):
    C2=C1/X C2: LOG(C2):
    C3=C2/X C3: LOG(C3):
    C4=C3/X C4: LOG(C4):
    C5=C4/X C5: LOG(C5):
    C6=C5/X C6: LOG(C6):
    C7=C6/X C7: LOG(C7):
    C8=C7/X C8: LOG(C8):
分子量计算器

分子量计算器

通过输入化合物的化学式来计算其分子量:

总分子量:g/mol

注:化学分子式大小写敏感。C10H16N2O2 c10h16n2o2

摩尔浓度计算器

质量 浓度 体积 分子量
计算

临床试验信息

NCT Number Recruitment interventions Conditions Sponsor/Collaborators Start Date Phases
NCT03512119 Completed Drug: Lumacaftor-Ivacaftor treatment Cystic Fibrosis Homozygous for Phe 508 Del CFTR|Glucose Intolerance or Newly Diagnosis Diabetes University Hospital Strasbourg France February 11 2016 --
NCT02514473 Completed Drug: VX-809|Drug: Placebo|Drug: VX-770 Cystic Fibrosis Vertex Pharmaceuticals Incorporated July 2015 Phase 3
NCT01899105 Completed Drug: lumacaftor|Drug: ivacaftor Cystic Fibrosis Vertex Pharmaceuticals Incorporated July 2013 Phase 1
NCT01897233 Completed Drug: Lumacaftor|Drug: Ivacaftor Cystic Fibrosis Vertex Pharmaceuticals Incorporated July 2013 Phase 3

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操作手册

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CFTR Signaling Pathway Map

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Cell Lines Assay Type Concentration Incubation Time Formulation Activity Description PMID