Palmitoyl protein thioesterase 1 (PPT1) is a lysosomal enzyme that removes fatty acyl groups of chain lengths C14–C18 from cysteine residues of modified proteins. From: Progress in Lipid Research, 2002 About this pageSet alert Also in subject areas: Medicine and Dentistry Neuroscience Pharm...
Infantile neuronal ceroid lipofuscinosis (INCL) is caused by palmitoyl protein thioesterase-1 (PPT1) deficiency. Although INCL patients show signs of abnormal neurotransmission, manifested by myoclonus and seizures, the molecular mechanisms by which PPT1 deficiency causes this abnormality remain obscure. ...
Here, we investigated the role in apoptosis of palmitoyl protein thioesterase 1 (PPT1), another lysosomal enzyme that depalmitoylates proteins. We show that transformed fibroblasts derived from patients with the infantile form of neuronal ceroid lipofuscinosis (INCL), a neurodegenerative disease due to...
PPT1/Palmitoyl-protein thioesterase 1Human 发布时间:2025/5/8 16:23:22 关注度:506 种 属:Human/Mouse/Rat 标 签:详询 规 格:10ug/50ug/500ug/1mg 缓 冲 液:0.01mol/L的PBS、pH:7.2、0.2%Procline-300。 表达宿主:E.coli/Cells 内毒素:经LAL检测验证,小于0.1ng/ul(0.1EU/ug) 纯化方法:亲和纯...
中文名称:棕榈酰蛋白硫酯酶1抗体 中文同义词:棕榈酰蛋白硫酯酶1抗体 英文名称:Palmitoyl protein thioesterase 1 antibody 英文同义词: CAS号: 分子式: 分子量:0 EINECS号: 信息错误报告 您的Email: 按国家浏览Palmitoyl protein thioesterase 1 antibody国外供应商中国供应商 ...
The defective gene behind the INCL disease, CLN1, encodes for palmitoyl protein thioesterase 1 (PPT1) [3]. It consists of 306 amino acids, including a signal sequence of 26 amino acids and three N-linked glycosylation sites. The enzyme is transported into lysosomes of non-neuronal cells by...
R. Protein depalmitoylases. Crit. Rev. Biochem. Mol. Biol. 53, 83–98 (2018). Article CAS PubMed Google Scholar Kong, E. et al. Dynamic palmitoylation links cytosol-membrane shuttling of acyl-protein thioesterase-1 and acyl-protein thioesterase-2 with that of proto-oncogene H-ras ...
(seeref.1) andChapter 16). These lipid thioesters may play a role in the neurodegeneration caused by the deficiency in palmitoyl-protein thioesterase, the defective enzyme in this disorder (2). In order to better characterize these thioesters, a method has been developed for their purification...
We synthesized a large number of novel, non-hydrolyzable competitive inhibitors of palmitoyl:protein thioesterase (PPT1) and showed that some could act as chemical chaperones. One inhibitor (CS38: βAGDap(Pal)VKIKK) was taken up by lymphoblasts from patients with mutations leading to the T75P...