323 related articles for article (PubMed ID: 20412078)
1. Improvement in lipid and protein trafficking in Niemann-Pick C1 cells by correction of a secondary enzyme defect.
Devlin C; Pipalia NH; Liao X; Schuchman EH; Maxfield FR; Tabas I
Traffic; 2010 May; 11(5):601-15. PubMed ID: 20412078
[TBL] [Abstract][Full Text] [Related]
2. Niemann-Pick C1 protein: obligatory roles for N-terminal domains and lysosomal targeting in cholesterol mobilization.
Watari H; Blanchette-Mackie EJ; Dwyer NK; Glick JM; Patel S; Neufeld EB; Brady RO; Pentchev PG; Strauss JF
Proc Natl Acad Sci U S A; 1999 Feb; 96(3):805-10. PubMed ID: 9927649
[TBL] [Abstract][Full Text] [Related]
3. Cholesterol trapping in Niemann-Pick disease type B fibroblasts can be relieved by expressing the phosphotyrosine binding domain of GULP.
Lee CY; Ruel I; Denis M; Genest J; Kiss RS
J Clin Lipidol; 2013; 7(2):153-64. PubMed ID: 23415435
[TBL] [Abstract][Full Text] [Related]
4. The Niemann-Pick C1 protein resides in a vesicular compartment linked to retrograde transport of multiple lysosomal cargo.
Neufeld EB; Wastney M; Patel S; Suresh S; Cooney AM; Dwyer NK; Roff CF; Ohno K; Morris JA; Carstea ED; Incardona JP; Strauss JF; Vanier MT; Patterson MC; Brady RO; Pentchev PG; Blanchette-Mackie EJ
J Biol Chem; 1999 Apr; 274(14):9627-35. PubMed ID: 10092649
[TBL] [Abstract][Full Text] [Related]
5. Reduction of TMEM97 increases NPC1 protein levels and restores cholesterol trafficking in Niemann-pick type C1 disease cells.
Ebrahimi-Fakhari D; Wahlster L; Bartz F; Werenbeck-Ueding J; Praggastis M; Zhang J; Joggerst-Thomalla B; Theiss S; Grimm D; Ory DS; Runz H
Hum Mol Genet; 2016 Aug; 25(16):3588-3599. PubMed ID: 27378690
[TBL] [Abstract][Full Text] [Related]
6. 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]
7. 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]
8. 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]
9. Posttranslational regulation of acid sphingomyelinase in niemann-pick type C1 fibroblasts and free cholesterol-enriched chinese hamster ovary cells.
Reagan JW; Hubbert ML; Shelness GS
J Biol Chem; 2000 Dec; 275(48):38104-10. PubMed ID: 10978332
[TBL] [Abstract][Full Text] [Related]
10. Beneficial effects of primidone in Niemann-Pick disease type C (NPC)-model cells and mice: Reduction of unesterified cholesterol levels in cells and extension of lifespan in mice.
Ashikawa H; Mogi H; Honda T; Nakamura H; Murayama T
Eur J Pharmacol; 2021 Apr; 896():173907. PubMed ID: 33503462
[TBL] [Abstract][Full Text] [Related]
11. 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]
12. Overview of clinical, molecular, and therapeutic features of Niemann-Pick disease (types A, B, and C): Focus on therapeutic approaches.
Hosseini K; Fallahi J; Razban V; Sirat RZ; Varasteh M; Tarhriz V
Cell Biochem Funct; 2024 Jun; 42(4):e4028. PubMed ID: 38715125
[TBL] [Abstract][Full Text] [Related]
13. Creation of a mouse model for non-neurological (type B) Niemann-Pick disease by stable, low level expression of lysosomal sphingomyelinase in the absence of secretory sphingomyelinase: relationship between brain intra-lysosomal enzyme activity and central nervous system function.
Marathe S; Miranda SR; Devlin C; Johns A; Kuriakose G; Williams KJ; Schuchman EH; Tabas I
Hum Mol Genet; 2000 Aug; 9(13):1967-76. PubMed ID: 10942425
[TBL] [Abstract][Full Text] [Related]
14. Localization of Niemann-Pick C1 protein in astrocytes: implications for neuronal degeneration in Niemann- Pick type C disease.
Patel SC; Suresh S; Kumar U; Hu CY; Cooney A; Blanchette-Mackie EJ; Neufeld EB; Patel RC; Brady RO; Patel YC; Pentchev PG; Ong WY
Proc Natl Acad Sci U S A; 1999 Feb; 96(4):1657-62. PubMed ID: 9990080
[TBL] [Abstract][Full Text] [Related]
15. 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]
16. Zn2+-stimulated sphingomyelinase is secreted by many cell types and is a product of the acid sphingomyelinase gene.
Schissel SL; Schuchman EH; Williams KJ; Tabas I
J Biol Chem; 1996 Aug; 271(31):18431-6. PubMed ID: 8702487
[TBL] [Abstract][Full Text] [Related]
17. Lipid storage disorders block lysosomal trafficking by inhibiting a TRP channel and lysosomal calcium release.
Shen D; Wang X; Li X; Zhang X; Yao Z; Dibble S; Dong XP; Yu T; Lieberman AP; Showalter HD; Xu H
Nat Commun; 2012 Mar; 3():731. PubMed ID: 22415822
[TBL] [Abstract][Full Text] [Related]
18. δ-Tocopherol reduces lipid accumulation in Niemann-Pick type C1 and Wolman cholesterol storage disorders.
Xu M; Liu K; Swaroop M; Porter FD; Sidhu R; Firnkes S; Ory DS; Marugan JJ; Xiao J; Southall N; Pavan WJ; Davidson C; Walkley SU; Remaley AT; Baxa U; Sun W; McKew JC; Austin CP; Zheng W
J Biol Chem; 2012 Nov; 287(47):39349-60. PubMed ID: 23035117
[TBL] [Abstract][Full Text] [Related]
19. Niemann-Pick type C mutations cause lipid traffic jam.
Liscum L
Traffic; 2000 Mar; 1(3):218-25. PubMed ID: 11208105
[TBL] [Abstract][Full Text] [Related]
20. 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]
[Next] [New Search]
