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P G Pentchev

Explore the profile of P G Pentchev including associated specialties, affiliations and a list of published articles. Areas
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Articles 127
Citations 3298
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Recent Articles
1.
Kidder L, Colarusso P, Stewart S, Levin I, Appel N, Lester D, et al.
J Biomed Opt . 2012 Sep; 4(1):7-13. PMID: 23015163
We have applied Fourier transform infrared (IR) spectroscopic imaging to the investigation of the neuropathologic effects of a genetic lipid storage disease, Niemann-Pick type C (NPC). Tissue sections both from...
2.
Ong W, Sundaram R, Huang E, Ghoshal S, Kumar U, Pentchev P, et al.
Neuroscience . 2004 Sep; 128(3):561-70. PMID: 15381285
Niemann-Pick disease type C (NP-C) is an inherited disorder that is characterized biochemically by cellular cholesterol and glycolipid storage, and clinically by progressive neurodegeneration. Most cases of NP-C are caused...
3.
Higaki K, Ninomiya H, Sugimoto Y, Suzuki T, Taniguchi M, Niwa H, et al.
J Biochem . 2001 Jun; 129(6):875-80. PMID: 11388901
Chinese hamster ovary cell mutants defective in the NPC1 gene (NPC1-trap) were generated by retrovirus-mediated gene trap mutagenesis from a parental cell line JP17 expressing an ecotropic retrovirus receptor. Insertion...
4.
Zhang M, Dwyer N, Love D, Cooney A, Comly M, Neufeld E, et al.
Proc Natl Acad Sci U S A . 2001 Apr; 98(8):4466-71. PMID: 11296289
Niemann-Pick type C1 (NPC1) disease results from a defect in the NPC1 protein and is characterized by a pathological accumulation of cholesterol and glycolipids in endocytic organelles. We followed the...
5.
Yamamoto T, Ninomiya H, Matsumoto M, Ohta Y, Nanba E, Tsutsumi Y, et al.
J Med Genet . 2001 Feb; 37(9):707-12. PMID: 11182931
No abstract available.
6.
Zhang M, Dwyer N, Neufeld E, Love D, Cooney A, Comly M, et al.
J Biol Chem . 2000 Oct; 276(5):3417-25. PMID: 11032830
The Niemann-Pick C1 (NPC1) protein and endocytosed low density lipoprotein (LDL)-derived cholesterol were shown to enrich separate subsets of vesicles containing lysosomal associated membrane protein 2. Localization of Rab7 in...
7.
Watari H, Blanchette-Mackie E, Dwyer N, Watari M, Burd C, Patel S, et al.
Exp Cell Res . 2000 Aug; 259(1):247-56. PMID: 10942596
Mutations in the NPC1 gene cause Niemann-Pick type C disease, which is characterized by the accumulation of free cholesterol and other lipids in lysosomes. The NPC1 glycoprotein is located in...
8.
Incardona J, Gaffield W, Lange Y, Cooney A, Pentchev P, Liu S, et al.
Dev Biol . 2000 Aug; 224(2):440-52. PMID: 10926779
Cyclopamine is a teratogenic steroidal alkaloid that causes cyclopia by blocking Sonic hedgehog (Shh) signal transduction. We have tested whether this activity of cyclopamine is related to disruption of cellular...
9.
Ong W, Hu C, Soh Y, Lim T, Pentchev P, Patel S
Neuroscience . 2000 Apr; 97(1):143-53. PMID: 10771346
Sterol regulatory element binding proteins are membrane-bound transcription factors that activate expression of several genes controlling cellular cholesterol and fatty acid homeostasis. The present study aimed to investigate the in...
10.
Liu Y, Wu Y, Wada R, Neufeld E, Mullin K, HOWARD A, et al.
Hum Mol Genet . 2000 Apr; 9(7):1087-92. PMID: 10767333
Niemann-Pick disease Type C (NP-C) is a progressive neurodegenerative disorder caused by mutations in the NPC1 gene and characterized by intracellular accumulation of cholesterol and sphingo-lipids. The major neuronal storage...