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Quoted phrase not found in phrase index: "Unesterified cholesterol accumulation in cultured fibroblasts"
Page 1
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. Pipalia NH, et al. J Lipid Res. 2021;62:100114. doi: 10.1016/j.jlr.2021.100114. Epub 2021 Sep 3. J Lipid Res. 2021. PMID: 34481829 Free PMC article.
We also observed that inhibition of HSP90 increased the expression of HSP70, and overexpression of HSP70 also reduced cholesterol storage in NPC1(I1061T) fibroblasts. ...The increase in other chaperones as a consequence of HSP90 improves folding of NPC1 prote …
We also observed that inhibition of HSP90 increased the expression of HSP70, and overexpression of HSP70 also reduced cholesterol …
Niemann-Pick disease type C: an update.
Vanier MT, Pentchev P, Rodriguez-Lafrasse C, Rousson R. Vanier MT, et al. J Inherit Metab Dis. 1991;14(4):580-95. doi: 10.1007/BF01797928. J Inherit Metab Dis. 1991. PMID: 1749223 Review.
Although the primary lesion is still unknown, studies conducted over the past six years have led to a breakthrough by showing that this disorder is characterized by unique abnormalities of intracellular translocation of exogenous cholesterol. In cultured fibrobla …
Although the primary lesion is still unknown, studies conducted over the past six years have led to a breakthrough by showing that this diso …
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. Pipalia NH, et al. Proc Natl Acad Sci U S A. 2011 Apr 5;108(14):5620-5. doi: 10.1073/pnas.1014890108. Epub 2011 Mar 21. Proc Natl Acad Sci U S A. 2011. PMID: 21436030 Free PMC article.
Niemann-Pick type C (NPC) disease is predominantly caused by mutations in the NPC1 protein that affect intracellular cholesterol trafficking and cause accumulation of unesterified cholesterol and other lipids in lysosomal storage organelles. ...LBH589 …
Niemann-Pick type C (NPC) disease is predominantly caused by mutations in the NPC1 protein that affect intracellular cholesterol traf …
Primary cilium alterations and expression changes of Patched1 proteins in niemann-pick type C disease.
Formichi P, Battisti C, De Santi MM, Guazzo R, Tripodi SA, Radi E, Rossi B, Tarquini E, Federico A. Formichi P, et al. J Cell Physiol. 2018 Jan;233(1):663-672. doi: 10.1002/jcp.25926. Epub 2017 May 19. J Cell Physiol. 2018. PMID: 28332184
Niemann-Pick type C disease (NPC) is a disorder characterized by abnormal intracellular accumulation of unesterified cholesterol and glycolipids. Two distinct disease-causing genes have been isolated, NPC1 and NPC2. ...We assessed the presence of NPC1 and Ptc …
Niemann-Pick type C disease (NPC) is a disorder characterized by abnormal intracellular accumulation of unesterified choles …
Altered vitamin E status in Niemann-Pick type C disease.
Ulatowski L, Parker R, Davidson C, Yanjanin N, Kelley TJ, Corey D, Atkinson J, Porter F, Arai H, Walkley SU, Manor D. Ulatowski L, et al. J Lipid Res. 2011 Jul;52(7):1400-10. doi: 10.1194/jlr.M015560. Epub 2011 May 5. J Lipid Res. 2011. PMID: 21550990 Free PMC article.
Niemann-Pick type C (NPC) disease is a lysosomal storage disorder caused by mutations in the NPC1 or NPC2 gene, which regulates lipid transport through the endocytic pathway. NPC disease is characterized by massive intracellular accumulation of unesterified chole …
Niemann-Pick type C (NPC) disease is a lysosomal storage disorder caused by mutations in the NPC1 or NPC2 gene, which regulates lipid transp …
Quantitative comparison of the efficacy of various compounds in lowering intracellular cholesterol levels in Niemann-Pick type C fibroblasts.
Wehrmann ZT, Hulett TW, Huegel KL, Vaughan KT, Wiest O, Helquist P, Goodson H. Wehrmann ZT, et al. PLoS One. 2012;7(10):e48561. doi: 10.1371/journal.pone.0048561. Epub 2012 Oct 29. PLoS One. 2012. PMID: 23144769 Free PMC article.
Niemann-Pick Type C disease (NPC) is a lethal, autosomal recessive disorder caused by mutations in the NPC1 and NPC2 cholesterol transport proteins. NPC's hallmark symptoms include an accumulation of unesterified cholesterol and other lipids in the lat …
Niemann-Pick Type C disease (NPC) is a lethal, autosomal recessive disorder caused by mutations in the NPC1 and NPC2 cholesterol tran …
Altered sensitivities to potential inhibitors of cholesterol biosynthesis in Niemann-Pick type C fibroblasts.
Ohno K, Nanba E, Nakano T, Inui K, Okada S, Takeshita K. Ohno K, et al. Cell Struct Funct. 1993 Aug;18(4):231-40. doi: 10.1247/csf.18.231. Cell Struct Funct. 1993. PMID: 8293500 Free article.
Cultured fibroblasts from patients with Niemann-Pick disease type C (NP-C) are characterized by the lysosomal accumulation of unesterified cholesterol and the inability of low-density lipoprotein (LDL) to stimulate cholesterol esterificat …
Cultured fibroblasts from patients with Niemann-Pick disease type C (NP-C) are characterized by the lysosomal accumulation …
Intracellular transport of cholesterol in type C Niemann-Pick fibroblasts.
Slotte JP, Hedström G, Bierman EL. Slotte JP, et al. Biochim Biophys Acta. 1989 Oct 17;1005(3):303-9. doi: 10.1016/0005-2760(89)90053-2. Biochim Biophys Acta. 1989. PMID: 2804059
This is relevant in light of the observations that NPC cells display a sluggish metabolism of LDL-derived cholesterol, a phenomenon which could be explained by a defective intracellular transport of cholesterol. ...Although NPC cells showed a relative accu …
This is relevant in light of the observations that NPC cells display a sluggish metabolism of LDL-derived cholesterol, a pheno …
Abnormal cholesterol metabolism in imipramine-treated fibroblast cultures. Similarities with Niemann-Pick type C disease.
Rodriguez-Lafrasse C, Rousson R, Bonnet J, Pentchev PG, Louisot P, Vanier MT. Rodriguez-Lafrasse C, et al. Biochim Biophys Acta. 1990 Apr 2;1043(2):123-8. doi: 10.1016/0005-2760(90)90284-5. Biochim Biophys Acta. 1990. PMID: 2317521
Addition of low-density lipoprotein (LDL) to cholesterol-deprived human skin fibroblast cultures treated by imipramine at a 20 microM concentration induced a significant intracellular accumulation of unesterified cholesterol. ...Treated c …
Addition of low-density lipoprotein (LDL) to cholesterol-deprived human skin fibroblast cultures treated by imipramine …
The intracellular transport of low density lipoprotein-derived cholesterol is defective in Niemann-Pick type C fibroblasts.
Liscum L, Ruggiero RM, Faust JR. Liscum L, et al. J Cell Biol. 1989 May;108(5):1625-36. doi: 10.1083/jcb.108.5.1625. J Cell Biol. 1989. PMID: 2715172 Free PMC article.
Niemann-Pick disease type C (NPC) is characterized by substantial intracellular accumulation of unesterified cholesterol. The accumulation of unesterified cholesterol in NPC fibroblasts cultured with low density lipoprotein …
Niemann-Pick disease type C (NPC) is characterized by substantial intracellular accumulation of unesterified cholesterol …
21 results