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Academic Journal

Cognition and anatomy of adult Niemann-Pick disease type C: Insights for the Alzheimer field.

  • Authors : Bergeron D; a Clinique Interdisciplinaire de Mémoire, Département des Sciences Neurologiques , CHU de Québec , Quebec City , Quebec , Canada.; b Faculté de Médecine , Université Laval , Quebec City , Quebec , Canada.

Subjects: Alzheimer Disease/Alzheimer Disease/Alzheimer Disease/*diagnosis ; Brain/Brain/Brain/*pathology ; Niemann-Pick Diseases/Niemann-Pick Diseases/Niemann-Pick Diseases/*complications

  • Source: Cognitive neuropsychology [Cogn Neuropsychol] 2018 May - Jun; Vol. 35 (3-4), pp. 209-222. Date of Electronic Publication: 2017 Jun 30.Publisher: Routledge Country of Publication: England NLM ID: 8411889 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1464-0627

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Academic Journal

Clinical, electrophysiological, and serum biochemical measures of progressive neurological and hepatic dysfunction in feline Niemann-Pick type C disease.

  • Authors : Vite CH; Department of Clinical Studies, University of Pennsylvania, School of Veterinary Medicine, Philadelphia, Pennsylvania 19104, USA. ; Ding W

Subjects: Brain/Brain/Brain/*physiopathology ; Cat Diseases/Cat Diseases/Cat Diseases/*physiopathology ; Liver/Liver/Liver/*physiopathology

  • Source: Pediatric research [Pediatr Res] 2008 Nov; Vol. 64 (5), pp. 544-9.Publisher: Nature Publishing Group Country of Publication: United States NLM ID: 0100714 Publication Model: Print Cited Medium: Internet ISSN:

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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

Combination brain and systemic injections of AAV provide maximal functional and survival benefits in the Niemann-Pick mouse.

  • Authors : Passini MA; Genzyme Corporation, Framingham, MA 01701, USA. ; Bu J

Subjects: Brain/Brain/Brain/*enzymology ; Brain/Brain/Brain/*pathology ; Dependovirus/Dependovirus/Dependovirus/*genetics

  • Source: Proceedings of the National Academy of Sciences of the United States of America [Proc Natl Acad Sci U S A] 2007 May 29; Vol. 104 (22), pp. 9505-10. Date of Electronic Publication: Publisher: National Academy of Sciences Country of Publication: United States NLM ID: 7505876 Publication Model: Print-Electronic Cited Medium: Print

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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

AAV vector-mediated correction of brain pathology in a mouse model of Niemann-Pick A disease.

  • Authors : Passini MA; Neuroscience, Genzyme Corporation, One Mountain Road, Framingham, MA 01701, USA. ; Macauley SL

Subjects: Disease Models, Animal* ; Genetic Therapy*; Brain/Brain/Brain/*pathology

  • Source: Molecular therapy : the journal of the American Society of Gene Therapy [Mol Ther] 2005 May; Vol. 11 (5), pp. 754-62.Publisher: Cell Press Country of Publication: United States NLM ID: 100890581 Publication Model: Print Cited Medium: Print ISSN: 1525-0016

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Academic Journal

Pathological cholesterol metabolism fails to modify electrophysiological properties of afflicted neurones in Niemann-Pick disease type C.

  • Authors : Deisz RA; Center of Anatomy, Charité, Institute of Integrative Neuroanatomy, Department of Clinical Cell and Neurobiology, Schumann Strasse 20-21, 10098 Berlin, Germany.; Meske V

Subjects: Brain/Brain/Brain/*metabolism ; Cell Membrane/Cell Membrane/Cell Membrane/*metabolism ; Cholesterol/Cholesterol/Cholesterol/*metabolism

  • Source: Neuroscience [Neuroscience] 2005; Vol. 130 (4), pp. 867-73.Publisher: Elsevier Science Country of Publication: United States NLM ID: 7605074 Publication Model: Print Cited Medium: Print ISSN: 0306-4522

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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

Intracerebral transplantation of adult mouse neural progenitor cells into the Niemann-Pick-A mouse leads to a marked decrease in lysosomal storage pathology.

Subjects: Stem Cell Transplantation*; Brain/Brain/Brain/*surgery ; Lysosomes/Lysosomes/Lysosomes/*pathology

  • Source: The Journal of neuroscience : the official journal of the Society for Neuroscience [J Neurosci] 2004 Nov 24; Vol. 24 (47), pp. 10642-51.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

Niemann pick type C disease as a model for defects in neurosteroidogenesis.

  • Authors : Mellon S; Department of Ob, Gyn, and Reproductive Sciences, University of California-San Francisco, 513 Parnassus Ave., San Francisco, CA 94143-0556, USA.; Gong W

Subjects: Brain/Brain/Brain/*metabolism ; Neurotransmitter Agents/Neurotransmitter Agents/Neurotransmitter Agents/*biosynthesis ; Niemann-Pick Diseases/Niemann-Pick Diseases/Niemann-Pick Diseases/*metabolism

  • Source: Endocrine research [Endocr Res] 2004 Nov; Vol. 30 (4), pp. 727-35.Publisher: Informa Healthcare Country of Publication: England NLM ID: 8408548 Publication Model: Print Cited Medium: Print ISSN: 0743-5800

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  • 1-10 of  128 results for ""NIEMANN-Pick diseases""