Peter G. Pentchev

Medicine · Health Sciences

47h-index9.8kcitations128works0.02yr avg
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Research Topics

Lysosomal Storage Disorders Research(93), Carbohydrate Chemistry and Synthesis(33), Glycogen Storage Diseases and Myoclonus(13), Biomedical Research and Pathophysiology(13), Glycosylation and Glycoproteins Research(12)

Publications127 total

Spreading the Wealth: Niemann-Pick Type C Proteins Bind and Transport Cholesterol
Cell Metabolism·2009· 4 citedOpen Access
Unraveling the sterol-trafficking defect in Niemann-Pick C disease
Proceedings of the National Academy of Sciences·2009· 20 citedOpen Access
Niemann–Pick C research from mouse to gene
Biochimica et Biophysica Acta (BBA) - Molecular and Cell Biology of Lipids·2004· 102 cited
Neuronal localization and association of Niemann Pick C2 protein (HE1/NPC2) with the postsynaptic density
Neuroscience·2004· 30 cited
Fitting a Mathematical Model to Biological Data: Intracellular Trafficking in Niemann-Pick C Disease
Advances in experimental medicine and biology·2003· 2 cited
Isolation of NPC1-Deficient Chinese Hamster Ovary Cell Mutants by Gene Trap Mutagenesis
The Journal of Biochemistry·2001· 47 cited
Cessation of rapid late endosomal tubulovesicular trafficking in Niemann–Pick type C1 disease
Proceedings of the National Academy of Sciences·2001· 146 citedOpen Access
Sterol-modulated Glycolipid Sorting Occurs in Niemann-Pick C1 Late Endosomes
Journal of Biological Chemistry·2001· 111 citedOpen Access
Cyclopamine Inhibition of Sonic Hedgehog Signal Transduction Is Not Mediated through Effects on Cholesterol Transport
Developmental Biology·2000· 98 cited
Determinants of NPC1 Expression and Action: Key Promoter Regions, Posttranscriptional Control, and the Importance of a “Cysteine-Rich” Loop
Experimental Cell Research·2000· 50 cited
Neuronal localization of sterol regulatory element binding protein-1 in the rodent and primate brain: a light and electron microscopic immunocytochemical study
Neuroscience·2000· 36 cited
NPC1-Containing Compartment of Human Granulosa-Lutein Cells: A Role in the Intracellular Trafficking of Cholesterol Supporting Steroidogenesis
Experimental Cell Research·2000· 51 cited
Niemann-Pick C1 Disease: The I1061T Substitution Is a Frequent Mutant Allele in Patients of Western European Descent and Correlates with a Classic Juvenile Phenotype
The American Journal of Human Genetics·1999· 204 citedOpen Access
The Genomic Organization and Polymorphism Analysis of the Human Niemann-Pick C1 Gene
Biochemical and Biophysical Research Communications·1999· 86 cited
NPC1 gene mutations in Japanese patients with Niemann-Pick disease type C
Human Genetics·1999· 87 cited
Mutations in the Leucine Zipper Motif and Sterol-sensing Domain Inactivate the Niemann-Pick C1 Glycoprotein
Journal of Biological Chemistry·1999· 133 citedOpen Access
The Niemann-Pick C1 Protein Resides in a Vesicular Compartment Linked to Retrograde Transport of Multiple Lysosomal Cargo
Journal of Biological Chemistry·1999· 374 citedOpen Access
Localization of Niemann–Pick C1 protein in astrocytes: Implications for neuronal degeneration in Niemann– Pick type C disease
Proceedings of the National Academy of Sciences·1999· 176 cited
Niemann-Pick C1 protein: Obligatory roles for N-terminal domains and lysosomal targeting in cholesterol mobilization
Proceedings of the National Academy of Sciences·1999· 163 cited
Cholesterol balance and metabolism in mice with loss of function of Niemann-Pick C protein
American Journal of Physiology-Endocrinology and Metabolism·1999· 114 cited
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Frequent Co-authors

Roscoe O. Brady(14), E. Joan Blanchette‐Mackie(11), Marcella Comly(10), Adele Cooney(9), Nancy K. Dwyer(9), Edward B. Neufeld(9), Shutish C. Patel(7), Jill A. Morris(6), Marie T. Vanier(6), Howard S. Kruth(6), Eugene D. Carstea(5), David A. Wenger(5), Jerome F. Strauss(5), Andrew E. Gal(5), M. T. Vanier(5), Claire Rodriguez‐Lafrasse(5), Hidemichi Watari(5), Kousaku Ohno(4), Shaun R. Patel(4), Calvin F. Roff(4)