| 351 |
12142809 | Niemann-pick disease type C in neonatal cholestasis at a North American Center. | 2002 Jul |
1 |
| 352 |
12364555 | Identification of a pharmaceutical compound that partially corrects the Niemann-Pick C phenotype in cultured cells. | 2002 Oct |
5 |
| 353 |
12398991 | Sterols and intracellular vesicular trafficking: lessons from the study of NPC1. | 2002 Nov |
5 |
| 354 |
11032830 | Sterol-modulated glycolipid sorting occurs in niemann-pick C1 late endosomes. | 2001 Feb 2 |
3 |
| 355 |
11202175 | Neurons in Niemann-Pick disease type C accumulate gangliosides as well as unesterified cholesterol and undergo dendritic and axonal alterations. | 2001 Jan |
2 |
| 356 |
11249924 | Quantified increases of cholesterol, total lipid and globotriaosylceramide in filipin-positive Niemann-Pick type C fibroblasts. | 2001 Mar |
3 |
| 357 |
11251074 | Dynamic movements of organelles containing Niemann-Pick C1 protein: NPC1 involvement in late endocytic events. | 2001 Mar |
2 |
| 358 |
11296289 | Cessation of rapid late endosomal tubulovesicular trafficking in Niemann-Pick type C1 disease. | 2001 Apr 10 |
1 |
| 359 |
11329376 | Depletion of rafts in late endocytic membranes is controlled by NPC1-dependent recycling of cholesterol to the plasma membrane. | 2001 May |
3 |
| 360 |
11349231 | Niemann-Pick C variant detection by altered sphingolipid trafficking and correlation with mutations within a specific domain of NPC1. | 2001 Jun |
2 |
| 361 |
11479732 | Niemann-Pick type C disease: NPC1 mutations associated with severe and mild cellular cholesterol trafficking alterations. | 2001 Jul |
2 |
| 362 |
11533723 | Multidrug permeases and subcellular cholesterol transport. | 2001 Sep |
1 |
| 363 |
10662671 | Mobilization of late-endosomal cholesterol is inhibited by Rab guanine nucleotide dissociation inhibitor. | 2000 Jan 27 |
5 |
| 364 |
10666334 | NPC1-containing compartment of human granulosa-lutein cells: a role in the intracellular trafficking of cholesterol supporting steroidogenesis. | 2000 Feb 25 |
1 |
| 365 |
10751394 | Cholesterol movement in Niemann-Pick type C cells and in cells treated with amphiphiles. | 2000 Jun 9 |
8 |
| 366 |
10783261 | Evidence for a Niemann-pick C (NPC) gene family: identification and characterization of NPC1L1. | 2000 Apr 15 |
3 |
| 367 |
10801441 | A model for niemann-pick type C disease in the nematode Caenorhabditis elegans. | 2000 May 4 |
2 |
| 368 |
10856720 | Intracellular cholesterol trafficking: role of the NPC1 protein. | 2000 Jun 26 |
1 |
| 369 |
10924274 | Deficient ferritin immunoreactivity in tissues from niemann-pick type C patients: extension of findings to fetal tissues, H and L ferritin isoforms, but also one case of the rare Niemann-Pick C2 complementation group. | 2000 Jul |
1 |
| 370 |
10926779 | Cyclopamine inhibition of Sonic hedgehog signal transduction is not mediated through effects on cholesterol transport. | 2000 Aug 15 |
2 |
| 371 |
10942596 | Determinants of NPC1 expression and action: key promoter regions, posttranscriptional control, and the importance of a "cysteine-rich" loop. | 2000 Aug 25 |
2 |
| 372 |
11001808 | The structure and function of the Niemann-Pick C1 protein. | 2000 Sep-Oct |
1 |
| 373 |
11013265 | Fate of endogenously synthesized cholesterol in Niemann-Pick type C1 cells. | 2000 Dec 29 |
5 |
| 374 |
11104205 | Niemann-Pick disease type C: two cases and an update. | 2000 Nov |
1 |
| 375 |
11208105 | Niemann-Pick type C mutations cause lipid traffic jam. | 2000 Mar |
2 |
| 376 |
9990080 | Localization of Niemann-Pick C1 protein in astrocytes: implications for neuronal degeneration in Niemann- Pick type C disease. | 1999 Feb 16 |
3 |
| 377 |
10092649 | The Niemann-Pick C1 protein resides in a vesicular compartment linked to retrograde transport of multiple lysosomal cargo. | 1999 Apr 2 |
2 |
| 378 |
10227680 | Lipid changes in Niemann-Pick disease type C brain: personal experience and review of the literature. | 1999 Apr |
4 |
| 379 |
10347764 | Effect of dimethylsulfoxide on sphingomyelinase activity and cholesterol metabolism in Niemann-Pick type C fibroblasts. | 1999 Jan |
2 |
| 380 |
10559883 | Late endosomal membranes rich in lysobisphosphatidic acid regulate cholesterol transport. | 1999 Jun |
3 |
| 381 |
10622400 | Progesterone metabolism in human fibroblasts is independent of P-glycoprotein levels and Niemann-Pick type C disease. | 1999 Sep-Oct |
1 |
| 382 |
9458174 | Recent advances in elucidating Niemann-Pick C disease. | 1998 Jan |
3 |
| 383 |
9533562 | Accumulation of cholesterol and GM2 ganglioside in cells cultured in the presence of progesterone: an implication for the basic defect in Niemann-Pick disease type C. | 1998 Jan |
6 |
| 384 |
9630707 | Decreased membrane fluidity and unsaturated fatty acids in Niemann-Pick disease type C fibroblasts. | 1998 Apr 28 |
3 |
| 385 |
9866822 | Niemann-Pick C disease: cholesterol handling gone awry. | 1998 Dec |
5 |
| 386 |
9060145 | Phenotypic and genetic heterogeneity in Niemann-Pick disease type C: current knowledge and practical implications. | 1997 Feb 14 |
5 |
| 387 |
9207099 | Substantial narrowing of the Niemann-Pick C candidate interval by yeast artificial chromosome complementation. | 1997 Jul 8 |
4 |
| 388 |
9211849 | Niemann-Pick C1 disease gene: homology to mediators of cholesterol homeostasis. | 1997 Jul 11 |
3 |
| 389 |
9458266 | Intracellular trafficking of the free cholesterol derived from LDL cholesteryl ester is defective in vivo in Niemann-Pick C disease: insights on normal metabolism of HDL and LDL gained from the NP-C mutation. | 1997 Dec |
6 |
| 390 |
7675237 | Psychosis as the initial manifestation of adult-onset Niemann-Pick disease type C. | 1995 Sep |
2 |
| 391 |
7754743 | Neurofibrillary tangles in Niemann-Pick disease type C. | 1995 |
5 |
| 392 |
7789012 | Marked heterogeneity in Niemann-Pick disease, type C. Clinical and ultrastructural findings. | 1995 Apr |
1 |
| 393 |
8204665 | Regulation of intracellular cholesterol metabolism is defective in lymphoblasts from Niemann-Pick type C and type D patients. | 1994 May 25 |
4 |
| 394 |
14731625 | The NP-C gene: a key to pathways of intracellular cholesterol transport. | 1994 Oct |
4 |
| 395 |
8423912 | The effect of cholesterol-lowering agents on hepatic and plasma cholesterol in Niemann-Pick disease type C. | 1993 Jan |
6 |
| 396 |
1543723 | Cultured fibroblasts from patients with Niemann-Pick disease type C and type D exhibit distinct defects in cholesterol esterification. | 1992 Feb 20 |
5 |
| 397 |
1716424 | Effects of drugs on cholesterol esterification in normal and Niemann-Pick type C fibroblasts: AY-9944, other cationic amphiphilic drugs and DMSO. | 1991 |
2 |
| 398 |
2590161 | Defective activity of acyl-CoA:cholesterol O-acyltransferase in Niemann-Pick type C and type D fibroblasts. | 1989 Sep 15 |
2 |
| 399 |
2688743 | Type C Niemann-Pick disease: dimethyl sulfoxide moderates abnormal LDL-cholesterol processing in mutant fibroblasts. | 1989 Nov 28 |
2 |
| 400 |
3378364 | Niemann-Pick disease group C: clinical variability and diagnosis based on defective cholesterol esterification. A collaborative study on 70 patients. | 1988 May |
2 |