329 related articles for article (PubMed ID: 33878344)
1. Lipid-mediated motor-adaptor sequestration impairs axonal lysosome delivery leading to autophagic stress and dystrophy in Niemann-Pick type C.
Roney JC; Li S; Farfel-Becker T; Huang N; Sun T; Xie Y; Cheng XT; Lin MY; Platt FM; Sheng ZH
Dev Cell; 2021 May; 56(10):1452-1468.e8. PubMed ID: 33878344
[TBL] [Abstract][Full Text] [Related]
2. Lipid-mediated impairment of axonal lysosome transport contributing to autophagic stress.
Roney JC; Li S; Farfel-Becker T; Huang N; Sun T; Xie Y; Cheng XT; Lin MY; Platt FM; Sheng ZH
Autophagy; 2021 Jul; 17(7):1796-1798. PubMed ID: 34085599
[TBL] [Abstract][Full Text] [Related]
3. β-Cyclodextrin-threaded biocleavable polyrotaxanes ameliorate impaired autophagic flux in Niemann-Pick type C disease.
Tamura A; Yui N
J Biol Chem; 2015 Apr; 290(15):9442-54. PubMed ID: 25713067
[TBL] [Abstract][Full Text] [Related]
4. NPC1-mTORC1 Signaling Couples Cholesterol Sensing to Organelle Homeostasis and Is a Targetable Pathway in Niemann-Pick Type C.
Davis OB; Shin HR; Lim CY; Wu EY; Kukurugya M; Maher CF; Perera RM; Ordonez MP; Zoncu R
Dev Cell; 2021 Feb; 56(3):260-276.e7. PubMed ID: 33308480
[TBL] [Abstract][Full Text] [Related]
5. Impaired proteolysis underlies autophagic dysfunction in Niemann-Pick type C disease.
Elrick MJ; Yu T; Chung C; Lieberman AP
Hum Mol Genet; 2012 Nov; 21(22):4876-87. PubMed ID: 22872701
[TBL] [Abstract][Full Text] [Related]
6. Intracellular cholesterol transport inhibition Impairs autophagy flux by decreasing autophagosome-lysosome fusion.
Maharjan Y; Dutta RK; Son J; Wei X; Park C; Kwon HM; Park R
Cell Commun Signal; 2022 Nov; 20(1):189. PubMed ID: 36434621
[TBL] [Abstract][Full Text] [Related]
7. Targeting defective sphingosine kinase 1 in Niemann-Pick type C disease with an activator mitigates cholesterol accumulation.
Newton J; Palladino END; Weigel C; Maceyka M; Gräler MH; Senkal CE; Enriz RD; Marvanova P; Jampilek J; Lima S; Milstien S; Spiegel S
J Biol Chem; 2020 Jul; 295(27):9121-9133. PubMed ID: 32385114
[TBL] [Abstract][Full Text] [Related]
8. Hydroxypropyl-beta and -gamma cyclodextrins rescue cholesterol accumulation in Niemann-Pick C1 mutant cell via lysosome-associated membrane protein 1.
Singhal A; Szente L; Hildreth JEK; Song B
Cell Death Dis; 2018 Oct; 9(10):1019. PubMed ID: 30282967
[TBL] [Abstract][Full Text] [Related]
9. Autophagic dysfunction in a lysosomal storage disorder due to impaired proteolysis.
Elrick MJ; Lieberman AP
Autophagy; 2013 Feb; 9(2):234-5. PubMed ID: 23086309
[TBL] [Abstract][Full Text] [Related]
10. 2-Hydroxypropyl-gamma-cyclodextrin overcomes NPC1 deficiency by enhancing lysosome-ER association and autophagy.
Singhal A; Krystofiak ES; Jerome WG; Song B
Sci Rep; 2020 May; 10(1):8663. PubMed ID: 32457374
[TBL] [Abstract][Full Text] [Related]
11. TDP-43 proteinopathy occurs independently of autophagic substrate accumulation and underlies nuclear defects in Niemann-Pick C disease.
Liu EA; Mori E; Hamasaki F; Lieberman AP
Neuropathol Appl Neurobiol; 2021 Dec; 47(7):1019-1032. PubMed ID: 34048071
[TBL] [Abstract][Full Text] [Related]
12. The pathogenesis of Niemann-Pick type C disease: a role for autophagy?
Pacheco CD; Lieberman AP
Expert Rev Mol Med; 2008 Sep; 10():e26. PubMed ID: 18782459
[TBL] [Abstract][Full Text] [Related]
13. Cholesterol accumulates in cell bodies, but is decreased in distal axons, of Niemann-Pick C1-deficient neurons.
Karten B; Vance DE; Campenot RB; Vance JE
J Neurochem; 2002 Dec; 83(5):1154-63. PubMed ID: 12437586
[TBL] [Abstract][Full Text] [Related]
14. Enrichment of NPC1-deficient cells with the lipid LBPA stimulates autophagy, improves lysosomal function, and reduces cholesterol storage.
Ilnytska O; Lai K; Gorshkov K; Schultz ML; Tran BN; Jeziorek M; Kunkel TJ; Azaria RD; McLoughlin HS; Waghalter M; Xu Y; Schlame M; Altan-Bonnet N; Zheng W; Lieberman AP; Dobrowolski R; Storch J
J Biol Chem; 2021 Jul; 297(1):100813. PubMed ID: 34023384
[TBL] [Abstract][Full Text] [Related]
15. Methyl-β-cyclodextrin restores impaired autophagy flux in Niemann-Pick C1-deficient cells through activation of AMPK.
Dai S; Dulcey AE; Hu X; Wassif CA; Porter FD; Austin CP; Ory DS; Marugan J; Zheng W
Autophagy; 2017 Aug; 13(8):1435-1451. PubMed ID: 28613987
[TBL] [Abstract][Full Text] [Related]
16. Impaired Retromer Function in Niemann-Pick Type C Disease Is Dependent on Intracellular Cholesterol Accumulation.
Dominko K; Rastija A; Sobocanec S; Vidatic L; Meglaj S; Lovincic Babic A; Hutter-Paier B; Colombo AV; Lichtenthaler SF; Tahirovic S; Hecimovic S
Int J Mol Sci; 2021 Dec; 22(24):. PubMed ID: 34948052
[TBL] [Abstract][Full Text] [Related]
17. In Vitro and In Vivo Evaluation of 6-O-α-Maltosyl-β-Cyclodextrin as a Potential Therapeutic Agent Against Niemann-Pick Disease Type C.
Yasmin N; Ishitsuka Y; Fukaura M; Yamada Y; Nakahara S; Ishii A; Kondo Y; Takeo T; Nakagata N; Motoyama K; Higashi T; Okada Y; Nishikawa J; Ichikawa A; Iohara D; Hirayama F; Higaki K; Ohno K; Matsuo M; Irie T
Int J Mol Sci; 2019 Mar; 20(5):. PubMed ID: 30845767
[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. Niemann-Pick type C disease: cellular pathology and pharmacotherapy.
Wheeler S; Sillence DJ
J Neurochem; 2020 Jun; 153(6):674-692. PubMed ID: 31608980
[TBL] [Abstract][Full Text] [Related]
20. Genistein Activates Transcription Factor EB and Corrects Niemann-Pick C Phenotype.
Argüello G; Balboa E; Tapia PJ; Castro J; Yañez MJ; Mattar P; Pulgar R; Zanlungo S
Int J Mol Sci; 2021 Apr; 22(8):. PubMed ID: 33921734
[TBL] [Abstract][Full Text] [Related]
[Next] [New Search]
