同样,MBTPS1-KO HaCaT细胞和Mbtps1-cKO小鼠中也观察到ETFA和ETFB的蛋白水平下降,除此之外,MBTPS1敲除不影响ETF的mRNA表达水平,但诱导ETF快速降解,这表明S1P可能维持了ETF的蛋白质稳定性。 与野生型S1P相比,突变型S1P与ETFA和ETFB的结合减少,这表明MBTPS1变异失去了与ETF形成三聚体复合物的能力。后续的分析表明,...
结果表明，S1P的缺失会损害线粒体呼吸和三羧酸循环（TCA），并导致线粒体代谢从氧化磷酸化转变为糖酵解。图2 S1P功能障碍破坏了ETF黄素化和稳定性4.核黄素疗法可减轻患者的炎性病变考虑到MBTPS1变异破坏了ETF的稳定性，研究人员提出用核黄素来逆转线粒体异常并治疗CAOP综合征。正如预期的那样，核黄素以浓度依赖...
同样,MBTPS1-KO HaCaT细胞和Mbtps1-cKO小鼠中也观察到ETFA和ETFB的蛋白水平下降,除此之外,MBTPS1敲除不影响ETF的mRNA表达水平,但诱导ETF快速降解,这表明S1P可能维持了ETF的蛋白质稳定性。 与野生型S1P相比,突变型S1P与ETFA和ETFB的结合减少,这表明MBTPS1变异失去了与ETF形成三聚体复合物的能力。后续的分析表明,...
蛋白别名:Endopeptidase S1P; membrane-bound transcription factor peptidase, site 1; membrane-bound transcription factor protease, site 1; Membrane-bound transcription factor site-1 protease; proprotein convertase subtilisin/kexin type 8; S1P endopeptidase; site-1 protease; SKI-1; Sterol-regulated luminal...
Membrane-bound transcription factor peptidase, site 1 (Mbtps1), also known as site-1 protease (S1P), cleaves several host and viral proteins. Host cell substrates include S1P itself, b-ZIP transcription factors such as the sterol regulatory element binding proteins (SREBP1 and SREBP2), ATF6,...
上海交通大学医学院附属新华医院姚志荣/李明/张思团队历经十余年研究,首次报告膜结合转录因子肽酶/位点1蛋白酶(MBTPS1/S1P)是CAOP综合征的致病基因,并阐明了MBTPS1突变的致病机理。他们还发现补充核黄素可改善CAOP综合征患者的炎性病变,为这种罕见皮肤病的治疗指明了方向。
上海玉博生物科技有限公司做为LSBIO代理商之产品LS-F65914-Mouse MBTPS1 / S1P (Custom) ELISA Kit - LS-F65914的产品销售公司,专业经营原装进口抗体(单克隆、多克隆)、重组蛋白、检测试剂盒、标准品、细胞因子、胎牛血清、细胞培养类等生物试剂以及耗材。
Variants in MBTPS1 (membrane‐bound transcription factor peptidase, site 1) encoding the protein convertase site‐1 protease (S1P) were recently reported in a single individual with skeletal dysplasia and elevated plasma lysosomal enzymes. Here, we report the second individual with this newly ...
Using PCR and RFLP analysis on a panel of genomic DNA from a mouse intersubspecific backcross, we have mapped the SK1-/S1P gene (locus symbol: Mbtps1) to the distal part of mouse chromosome 8, in a region that exhibits synteny homology to the human chromosome 16q24 region where its ...
上海玉博生物科技有限公司做为LSBIO代理商之产品LS-F65914-Mouse MBTPS1 / S1P (Custom) ELISA Kit - LS-F65914的产品销售公司,专业经营原装进口抗体(单克隆、多克隆)、重组蛋白、检测试剂盒、标准品、细胞因子、胎牛血清、细胞培养类等生物试剂以及耗材。