190 related articles for article (PubMed ID: 34151204)
1. Inhibition of Histone Deacetylases 1, 2, and 3 Enhances Clearance of Cholesterol Accumulation in Niemann-Pick C1 Fibroblasts.
Cruz DL; Pipalia N; Mao S; Gadi D; Liu G; Grigalunas M; O'Neill M; Quinn TR; Kipper A; Ekebergh A; Dimmling A; Gartner C; Melancon BJ; Wagner FF; Holson E; Helquist P; Wiest O; Maxfield FR
ACS Pharmacol Transl Sci; 2021 Jun; 4(3):1136-1148. PubMed ID: 34151204
[TBL] [Abstract][Full Text] [Related]
2. Histone deacetylase inhibitors correct the cholesterol storage defect in most Niemann-Pick C1 mutant cells.
Pipalia NH; Subramanian K; Mao S; Ralph H; Hutt DM; Scott SM; Balch WE; Maxfield FR
J Lipid Res; 2017 Apr; 58(4):695-708. PubMed ID: 28193631
[TBL] [Abstract][Full Text] [Related]
3. Histone deacetylase inhibitor treatment dramatically reduces cholesterol accumulation in Niemann-Pick type C1 mutant human fibroblasts.
Pipalia NH; Cosner CC; Huang A; Chatterjee A; Bourbon P; Farley N; Helquist P; Wiest O; Maxfield FR
Proc Natl Acad Sci U S A; 2011 Apr; 108(14):5620-5. PubMed ID: 21436030
[TBL] [Abstract][Full Text] [Related]
4. HSP90 inhibitors reduce cholesterol storage in Niemann-Pick type C1 mutant fibroblasts.
Pipalia NH; Saad SZ; Subramanian K; Cross A; Al-Motawa A; Garg K; Blagg BSJ; Neckers L; Helquist P; Wiest O; Ory DS; Maxfield FR
J Lipid Res; 2021; 62():100114. PubMed ID: 34481829
[TBL] [Abstract][Full Text] [Related]
5. Quantitative Analysis of the Proteome Response to the Histone Deacetylase Inhibitor (HDACi) Vorinostat in Niemann-Pick Type C1 disease.
Subramanian K; Rauniyar N; Lavalleé-Adam M; Yates JR; Balch WE
Mol Cell Proteomics; 2017 Nov; 16(11):1938-1957. PubMed ID: 28860124
[TBL] [Abstract][Full Text] [Related]
6. Correction of Niemann-Pick type C1 trafficking and activity with the histone deacetylase inhibitor valproic acid.
Subramanian K; Hutt DM; Scott SM; Gupta V; Mao S; Balch WE
J Biol Chem; 2020 Jun; 295(23):8017-8035. PubMed ID: 32354745
[TBL] [Abstract][Full Text] [Related]
7. ABCA1-dependent mobilization of lysosomal cholesterol requires functional Niemann-Pick C2 but not Niemann-Pick C1 protein.
Boadu E; Nelson RC; Francis GA
Biochim Biophys Acta; 2012 Mar; 1821(3):396-404. PubMed ID: 22179027
[TBL] [Abstract][Full Text] [Related]
8. Different Trafficking Phenotypes of Niemann-Pick C1 Gene Mutations Correlate with Various Alterations in Lipid Storage, Membrane Composition and Miglustat Amenability.
Brogden G; Shammas H; Walters F; Maalouf K; Das AM; Naim HY; Rizk S
Int J Mol Sci; 2020 Mar; 21(6):. PubMed ID: 32204338
[TBL] [Abstract][Full Text] [Related]
9. Normalization of Hepatic Homeostasis in the
Munkacsi AB; Hammond N; Schneider RT; Senanayake DS; Higaki K; Lagutin K; Bloor SJ; Ory DS; Maue RA; Chen FW; Hernandez-Ono A; Dahlson N; Repa JJ; Ginsberg HN; Ioannou YA; Sturley SL
J Biol Chem; 2017 Mar; 292(11):4395-4410. PubMed ID: 28031458
[TBL] [Abstract][Full Text] [Related]
10. FTY720/fingolimod increases NPC1 and NPC2 expression and reduces cholesterol and sphingolipid accumulation in Niemann-Pick type C mutant fibroblasts.
Newton J; Hait NC; Maceyka M; Colaco A; Maczis M; Wassif CA; Cougnoux A; Porter FD; Milstien S; Platt N; Platt FM; Spiegel S
FASEB J; 2017 Apr; 31(4):1719-1730. PubMed ID: 28082351
[TBL] [Abstract][Full Text] [Related]
11. Assessment of FDA-Approved Drugs as a Therapeutic Approach for Niemann-Pick Disease Type C1 Using Patient-Specific iPSC-Based Model Systems.
Völkner C; Pantoom S; Liedtke M; Lukas J; Hermann A; Frech MJ
Cells; 2022 Jan; 11(3):. PubMed ID: 35159129
[TBL] [Abstract][Full Text] [Related]
12. Niemann-Pick C variant detection by altered sphingolipid trafficking and correlation with mutations within a specific domain of NPC1.
Sun X; Marks DL; Park WD; Wheatley CL; Puri V; O'Brien JF; Kraft DL; Lundquist PA; Patterson MC; Pagano RE; Snow K
Am J Hum Genet; 2001 Jun; 68(6):1361-72. PubMed ID: 11349231
[TBL] [Abstract][Full Text] [Related]
13. A murine Niemann-Pick C1 I1061T knock-in model recapitulates the pathological features of the most prevalent human disease allele.
Praggastis M; Tortelli B; Zhang J; Fujiwara H; Sidhu R; Chacko A; Chen Z; Chung C; Lieberman AP; Sikora J; Davidson C; Walkley SU; Pipalia NH; Maxfield FR; Schaffer JE; Ory DS
J Neurosci; 2015 May; 35(21):8091-106. PubMed ID: 26019327
[TBL] [Abstract][Full Text] [Related]
14. Niemann-Pick type C1 I1061T mutant encodes a functional protein that is selected for endoplasmic reticulum-associated degradation due to protein misfolding.
Gelsthorpe ME; Baumann N; Millard E; Gale SE; Langmade SJ; Schaffer JE; Ory DS
J Biol Chem; 2008 Mar; 283(13):8229-36. PubMed ID: 18216017
[TBL] [Abstract][Full Text] [Related]
15. Differential trafficking of the Niemann-Pick C1 and 2 proteins highlights distinct roles in late endocytic lipid trafficking.
Zhang M; Sun M; Dwyer NK; Comly ME; Patel SC; Sundaram R; Hanover JA; Blanchette-Mackie EJ
Acta Paediatr Suppl; 2003 Dec; 92(443):63-73; discussion 45. PubMed ID: 14989468
[TBL] [Abstract][Full Text] [Related]
16. Mutations in the leucine zipper motif and sterol-sensing domain inactivate the Niemann-Pick C1 glycoprotein.
Watari H; Blanchette-Mackie EJ; Dwyer NK; Watari M; Neufeld EB; Patel S; Pentchev PG; Strauss JF
J Biol Chem; 1999 Jul; 274(31):21861-6. PubMed ID: 10419504
[TBL] [Abstract][Full Text] [Related]
17. An induced pluripotent stem cell line (TRNDi001-D) from a Niemann-Pick disease type C1 (NPC1) patient carrying a homozygous p. I1061T (c. 3182T>C) mutation in the NPC1 gene.
Li R; Pradhan M; Xu M; Roeder A; Beers J; Zou J; Liu C; Porter FD; Zheng W
Stem Cell Res; 2020 Apr; 44():101737. PubMed ID: 32114296
[TBL] [Abstract][Full Text] [Related]
18. A human iPSC-derived inducible neuronal model of Niemann-Pick disease, type C1.
Prabhu AV; Kang I; De Pace R; Wassif CA; Fujiwara H; Kell P; Jiang X; Ory DS; Bonifacino JS; Ward ME; Porter FD
BMC Biol; 2021 Oct; 19(1):218. PubMed ID: 34592985
[TBL] [Abstract][Full Text] [Related]
19. Transcript, protein, metabolite and cellular studies in skin fibroblasts demonstrate variable pathogenic impacts of NPC1 mutations.
Musalkova D; Majer F; Kuchar L; Luksan O; Asfaw B; Vlaskova H; Storkanova G; Reboun M; Poupetova H; Jahnova H; Hulkova H; Ledvinova J; Dvorakova L; Sikora J; Jirsa M; Vanier MT; Hrebicek M
Orphanet J Rare Dis; 2020 Apr; 15(1):85. PubMed ID: 32248828
[TBL] [Abstract][Full Text] [Related]
20. Ryanodine receptor antagonists adapt NPC1 proteostasis to ameliorate lipid storage in Niemann-Pick type C disease fibroblasts.
Yu T; Chung C; Shen D; Xu H; Lieberman AP
Hum Mol Genet; 2012 Jul; 21(14):3205-14. PubMed ID: 22505584
[TBL] [Abstract][Full Text] [Related]
[Next] [New Search]