Arimoclomol increases the transcription of lysosomal genes, including NPC1 and NPC2, to facilitate lysosomal function

被引:2
|
作者
Petersen, Nikolaj H. T. [1 ]
Klein, Pontus [1 ]
Valacca, Cristina [1 ]
Bie, Anne [1 ]
Magnoni, Raffaella [1 ]
Pedersen, Marianne T. [1 ]
Lampp, Andrea C. [1 ]
Fog, Cathrine K. [1 ]
Kirkegaard, Thomas T. [1 ]
机构
[1] Orphazyme, Copenhagen, Denmark
关键词
D O I
10.1016/j.ymgme.2021.11.251
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
236
引用
收藏
页码:S96 / S96
页数:1
相关论文
共 50 条
  • [21] Physiological and Coordinate Downregulation of the NPC1 and NPC2 Genes Are Associated With the Sequestration of LDL-Derived Cholesterol Within Endocytic Compartments
    Jelinek, David
    Patrick, Sarah Mount
    Kitt, Khameeka N.
    Chan, Teddy
    Francis, Gordon A.
    Garver, William S.
    JOURNAL OF CELLULAR BIOCHEMISTRY, 2009, 108 (05) : 1102 - 1116
  • [22] Clues to the mechanism of cholesterol transfer from the structure of NPC1 middle lumenal domain bound to NPC2
    Li, Xiaochun
    Saha, Piyali
    Li, Jian
    Blobel, Gunter
    Pfeffer, Suzanne R.
    PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA, 2016, 113 (36) : 10079 - 10084
  • [23] NPC1 and NPC2 gene analysis in Serbian patients with Niemann-Pick disease type C
    Brankovic, M.
    Kresojevic, N.
    Marjanovic, A.
    Novakovic, I.
    Kostic, V.
    EUROPEAN JOURNAL OF HUMAN GENETICS, 2018, 26 : 406 - 406
  • [24] Computational Tools Unravel Putative Sterol Binding Sites in the Lysosomal NPC1 Protein
    Elghobashi-Meinhardt, Nadia
    JOURNAL OF CHEMICAL INFORMATION AND MODELING, 2019, 59 (05) : 2432 - 2441
  • [25] NPC1 regulates the distribution of phosphatidylinositol 4-kinases at Golgi and lysosomal membranes
    Kutchukian, Candice
    Vivas, Oscar
    Casas, Maria
    Jones, Julia G.
    Tiscione, Scott A.
    Simo, Sergi
    Ory, Daniel S.
    Dixon, Rose E.
    Dickson, Eamonn J.
    EMBO JOURNAL, 2021, 40 (13):
  • [26] Structure of glycosylated NPC1 luminal domain C reveals insights into NPC2 and Ebola virus interactions
    Zhao, Yuguang
    Ren, Jingshan
    Harlos, Karl
    Stuart, David I.
    FEBS LETTERS, 2016, 590 (05) : 605 - 612
  • [27] Focusing on the Abnormal Events of NPC1, NPC2, and NPC1L1 in Pan-Cancer and Further Constructing LUAD and KICH Prediction Models
    Chen, Keheng
    Zhang, Xin
    Sun, Guangyu
    Fang, Zhichao
    Liao, Lusheng
    Zhong, Yanping
    Huang, Fengdie
    Dong, Mingyou
    Luo, Shihua
    JOURNAL OF PROTEOME RESEARCH, 2023, 23 (01) : 449 - 464
  • [28] Concerted regulation of NPC2 binding to endosomal/lysosomal membranes by bis(monoacylglycero)phosphate and sphingomyelin
    Enkavi, Giray
    Mikkolainen, Heikki
    Gungor, Burcin
    Ikonen, Elina
    Vattulainen, Ilpo
    PLOS COMPUTATIONAL BIOLOGY, 2017, 13 (10)
  • [29] Defective endocytic trafficking of NPC1 and NPC2 underlying infantile Niemann-Pick type C disease
    Blom, TS
    Linder, MD
    Snow, K
    Pihko, H
    Hess, MW
    Jokitalo, E
    Veckman, V
    Syvänen, AC
    Ikonen, E
    HUMAN MOLECULAR GENETICS, 2003, 12 (03) : 257 - 272
  • [30] NPC2 facilitates bidirectional transfer of cholesterol between NPC1 and lipid bilayers, a step in cholesterol egress from lysosomes
    Infante, Rodney E.
    Wang, Michael L.
    Radhakrishnan, Arun
    Kwon, Hyock Joo
    Brown, Michael S.
    Goldstein, Joseph L.
    PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA, 2008, 105 (40) : 15287 - 15292