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Academic Journal

Niemann-Pick C1 mice, a model of "juvenile Alzheimer's disease", with normal gene expression in neurons and fibrillary astrocytes show long term survival and delayed neurodegeneration.

  • Authors : Borbon I; Department of Pediatrics, University of Arizona, Tucson, AZ 85724-5073, USA.; Totenhagen J

Subjects: Alzheimer Disease/Alzheimer Disease/Alzheimer Disease/*genetics ; Astrocytes/Astrocytes/Astrocytes/*pathology ; Nerve Degeneration/Nerve Degeneration/Nerve Degeneration/*genetics

  • Source: Journal of Alzheimer's disease : JAD [J Alzheimers Dis] 2012; Vol. 30 (4), pp. 875-87.Publisher: IOS Press Country of Publication: Netherlands NLM ID: 9814863 Publication Model: Print Cited Medium: Internet ISSN: 1875-8908

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Academic Journal

Defects of synaptic vesicle turnover at excitatory and inhibitory synapses in Niemann-Pick C1-deficient neurons.

  • Authors : Xu S; Department of Neurobiology, Institute of Neuroscience, Zhejiang University School of Medicine, Hangzhou, Zhejiang, PR China.; Zhou S

Subjects: Brain/Brain/Brain/*metabolism ; Neurons/Neurons/Neurons/*metabolism ; Niemann-Pick Diseases/Niemann-Pick Diseases/Niemann-Pick Diseases/*metabolism

  • Source: Neuroscience [Neuroscience] 2010 May 19; Vol. 167 (3), pp. 608-20. Date of Electronic Publication: 2010 Feb 16.Publisher: Elsevier Science Country of Publication: United States NLM ID: 7605074 Publication Model: Print-Electronic Cited Medium: Internet ISSN:

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Academic Journal

Liver X receptor activation enhances cholesterol loss from the brain, decreases neuroinflammation, and increases survival of the NPC1 mouse.

  • Authors : Repa JJ; Department of Internal Medicine, University of Texas Southwestern Medical School, Dallas, Texas 75390-9151, USA. ; Li H

Subjects: Niemann-Pick Diseases*/Niemann-Pick Diseases*/Niemann-Pick Diseases*/diet therapy ; Niemann-Pick Diseases*/Niemann-Pick Diseases*/Niemann-Pick Diseases*/genetics ; Niemann-Pick Diseases*/Niemann-Pick Diseases*/Niemann-Pick Diseases*/mortality

  • Source: The Journal of neuroscience : the official journal of the Society for Neuroscience [J Neurosci] 2007 Dec 26; Vol. 27 (52), pp. 14470-80.Publisher: Society for Neuroscience Country of Publication: United States NLM ID: 8102140 Publication Model: Print Cited Medium: Internet ISSN:

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Academic Journal

Genes, environment, and lifespan: new insights into Niemann-Pick C disease.

  • Authors : Erickson SK; University of California, San Francisco, CA, USA.

Subjects: Niemann-Pick Diseases/Niemann-Pick Diseases/Niemann-Pick Diseases/*etiology ; Proteins/Proteins/Proteins/*genetics; Animals

  • Source: Journal of lipid research [J Lipid Res] 2008 Mar; Vol. 49 (3), pp. 497-8. Date of Electronic Publication: 2008 Jan 16.Publisher: Elsevier Country of Publication: United States NLM ID: 0376606 Publication Model: Print-Electronic Cited Medium: Print ISSN: 0022-2275

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Academic Journal

Defective insulin receptor activation and altered lipid rafts in Niemann-Pick type C disease hepatocytes.

  • Authors : Vainio S; National Public Health Institute, Helsinki, Finland.; Bykov I

Subjects: Hepatocytes/Hepatocytes/Hepatocytes/*metabolism ; Membrane Microdomains/Membrane Microdomains/Membrane Microdomains/*metabolism ; Niemann-Pick Diseases/Niemann-Pick Diseases/Niemann-Pick Diseases/*metabolism

  • Source: The Biochemical journal [Biochem J] 2005 Nov 01; Vol. 391 (Pt 3), pp. 465-72.Publisher: Published by Portland Press on behalf of the Biochemical Society Country of Publication: England NLM ID: 2984726R Publication Model: Print Cited

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Academic Journal

Cholesterol accumulation and liver cell death in mice with Niemann-Pick type C disease.

  • Authors : Beltroy EP; Department of Pediatrics, University of Texas Southwestern Medical School, Dallas, TX 75390-8887, USA.; Richardson JA

Subjects: Cholesterol/Cholesterol/Cholesterol/*metabolism ; Liver/Liver/Liver/*metabolism ; Liver/Liver/Liver/*pathology

  • Source: Hepatology (Baltimore, Md.) [Hepatology] 2005 Oct; Vol. 42 (4), pp. 886-93.Publisher: Wolters Kluwer Health, Inc Country of Publication: United States NLM ID: 8302946 Publication Model: Print Cited Medium: Print ISSN:

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Academic Journal

Cholesterol accumulation is associated with lysosomal dysfunction and autophagic stress in Npc1 -/- mouse brain.

  • Authors : Liao G; Department of Basic Medical Sciences, College of Osteopathic Medicine of the Pacific, Western University of Health Sciences, Pomona, CA 91766-1854, USA.; Yao Y

Subjects: Autophagy*; Brain/Brain/Brain/*metabolism ; Cholesterol/Cholesterol/Cholesterol/*metabolism

  • Source: The American journal of pathology [Am J Pathol] 2007 Sep; Vol. 171 (3), pp. 962-75. Date of Electronic Publication: 2007 Jul 13.Publisher: Elsevier Country of Publication: United States NLM ID: 0370502 Publication Model: Print-Electronic Cited Medium: Print ISSN: 0002-9440

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Academic Journal

Characterization of liver disease and lipid metabolism in the Niemann-Pick C1 mouse.

  • Authors : Garver WS; Department of Pediatrics, The University of Arizona, Tucson, AZ 85724, USA. ; Jelinek D

Subjects: Lipid Metabolism* ; Liver Diseases*/Liver Diseases*/Liver Diseases*/metabolism ; Liver Diseases*/Liver Diseases*/Liver Diseases*/physiopathology

  • Source: Journal of cellular biochemistry [J Cell Biochem] 2007 May 15; Vol. 101 (2), pp. 498-516.Publisher: Wiley-Liss Country of Publication: United States NLM ID: 8205768 Publication Model: Print Cited Medium: Print ISSN: 0730-2312 (Print)

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Academic Journal

Cyclin-dependent kinase inhibitors attenuate protein hyperphosphorylation, cytoskeletal lesion formation, and motor defects in Niemann-Pick Type C mice.

  • Authors : Zhang M; Department of Pathology, University of Washington, Seattle, Washington, USA.; Li J

Subjects: Cyclin-Dependent Kinases/Cyclin-Dependent Kinases/Cyclin-Dependent Kinases/*antagonists & inhibitors ; Cytoskeleton/Cytoskeleton/Cytoskeleton/*pathology ; Enzyme Inhibitors/Enzyme Inhibitors/Enzyme Inhibitors/*pharmacology

  • Source: The American journal of pathology [Am J Pathol] 2004 Sep; Vol. 165 (3), pp. 843-53.Publisher: Elsevier Country of Publication: United States NLM ID: 0370502 Publication Model: Print Cited Medium: Print ISSN: 0002-9440 (Print)

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Academic Journal

Sperm defects in mice lacking a functional Niemann-Pick C1 protein.

  • Authors : Fan J; Department of Biological Sciences, Marshall University, Huntington, WV 25755, USA.; Akabane H

Subjects: Infertility, Male/Infertility, Male/Infertility, Male/*pathology ; Niemann-Pick Diseases/Niemann-Pick Diseases/Niemann-Pick Diseases/*pathology ; Proteins/Proteins/Proteins/*genetics

  • Source: Molecular reproduction and development [Mol Reprod Dev] 2006 Oct; Vol. 73 (10), pp. 1284-91.Publisher: Wiley-Liss Country of Publication: United States NLM ID: 8903333 Publication Model: Print Cited Medium: Print ISSN: 1040-452X (Print)

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  • 1-10 of  142 results for ""Niemann-Pick Diseases""