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Search results 201 to 300 out of 584 for Npc1

0.041s
Type Details Score
Publication
First Author: Suzuki M
Year: 2007
Journal: J Neurosci
Title: Endosomal accumulation of Toll-like receptor 4 causes constitutive secretion of cytokines and activation of signal transducers and activators of transcription in Niemann-Pick disease type C (NPC) fibroblasts: a potential basis for glial cell activation in the NPC brain.
Volume: 27
Issue: 8
Pages: 1879-91
Publication
First Author: Cawley NX
Year: 2020
Journal: PLoS One
Title: Abnormal LAMP1 glycosylation may play a role in Niemann-Pick disease, type C pathology.
Volume: 15
Issue: 1
Pages: e0227829
Publication
First Author: Boland B
Year: 2010
Journal: J Biol Chem
Title: Macroautophagy is not directly involved in the metabolism of amyloid precursor protein.
Volume: 285
Issue: 48
Pages: 37415-26
Publication
First Author: Zhou S
Year: 2011
Journal: Am J Pathol
Title: Endosomal/lysosomal processing of gangliosides affects neuronal cholesterol sequestration in Niemann-Pick disease type C.
Volume: 179
Issue: 2
Pages: 890-902
Publication
First Author: Qin Q
Year: 2010
Journal: PLoS One
Title: Cholesterol Perturbation in Mice Results in p53 Degradation and Axonal Pathology through p38 MAPK and Mdm2 Activation.
Volume: 5
Issue: 4
Pages: e9999
Gene
Type: gene
Organism: frog, African clawed
Gene
Type: gene
Organism: frog, African clawed
Publication
First Author: Causevic M
Year: 2018
Journal: PLoS One
Title: BACE1-cleavage of Sez6 and Sez6L is elevated in Niemann-Pick type C disease mouse brains.
Volume: 13
Issue: 7
Pages: e0200344
Publication
First Author: Wang MD
Year: 2007
Journal: J Biol Chem
Title: Differential regulation of ATP binding cassette protein A1 expression and ApoA-I lipidation by Niemann-Pick type C1 in murine hepatocytes and macrophages.
Volume: 282
Issue: 31
Pages: 22525-33
Publication
First Author: Chu TT
Year: 2021
Journal: Nature
Title: Tonic prime-boost of STING signalling mediates Niemann-Pick disease type C.
Volume: 596
Issue: 7873
Pages: 570-575
Publication
First Author: Quan G
Year: 2003
Journal: Brain Res Dev Brain Res
Title: Ontogenesis and regulation of cholesterol metabolism in the central nervous system of the mouse.
Volume: 146
Issue: 1-2
Pages: 87-98
Publication
First Author: Ishibashi M
Year: 2010
Journal: J Lipid Res
Title: Reduced VLDL clearance in Apoe(-/-)Npc1(-/-) mice is associated with increased Pcsk9 and Idol expression and decreased hepatic LDL-receptor levels.
Volume: 51
Issue: 9
Pages: 2655-63
Publication
First Author: Garver WS
Year: 2000
Journal: J Lipid Res
Title: Localization of the murine Niemann-Pick C1 protein to two distinct intracellular compartments.
Volume: 41
Issue: 5
Pages: 673-87
Protein Coding Gene
Type: protein_coding_gene
Organism: Mus caroli
Protein Coding Gene
Type: protein_coding_gene
Organism: mouse, laboratory
Protein Coding Gene
Type: protein_coding_gene
Organism: mouse, laboratory
Protein Coding Gene
Type: protein_coding_gene
Organism: mouse, laboratory
Protein Coding Gene
Type: protein_coding_gene
Organism: mouse, laboratory
Protein Coding Gene
Type: protein_coding_gene
Organism: mouse, laboratory
Protein Coding Gene
Type: protein_coding_gene
Organism: mouse, laboratory
Protein Coding Gene
Type: protein_coding_gene
Organism: mouse, laboratory
Protein Coding Gene
Type: protein_coding_gene
Organism: mouse, laboratory
Protein Coding Gene
Type: protein_coding_gene
Organism: mouse, laboratory
Protein Coding Gene
Type: protein_coding_gene
Organism: mouse, laboratory
Protein Coding Gene
Type: protein_coding_gene
Organism: mouse, laboratory
Protein Coding Gene
Type: protein_coding_gene
Organism: mouse, laboratory
Protein Coding Gene
Type: protein_coding_gene
Organism: mouse, laboratory
Protein Coding Gene
Type: protein_coding_gene
Organism: mouse, laboratory
Protein Coding Gene
Type: protein_coding_gene
Organism: mouse, laboratory
Protein Coding Gene
Type: protein_coding_gene
Organism: mouse, laboratory
Protein Coding Gene
Type: protein_coding_gene
Organism: Mus pahari
Protein Coding Gene
Type: protein_coding_gene
Organism: Mus spretus
Publication
First Author: Miyawaki S
Year: 1982
Journal: J Hered
Title: Sphingomyelinosis, a new mutation in the mouse: a model of Niemann-Pick disease in humans.
Volume: 73
Issue: 4
Pages: 257-63
Publication
First Author: Akaboshi S
Year: 1997
Journal: Hum Genet
Title: A C57BL/KsJ mouse model of Niemann-Pick disease (spm) belongs to the same complementation group as the major childhood type of Niemann-Pick disease type C.
Volume: 99
Issue: 3
Pages: 350-3
Publication
First Author: Ohno K
Year: 1992
Journal: Cell Struct Funct
Title: A cell line derived from sphingomyelinosis mouse shows alterations in intracellular cholesterol metabolism similar to those in type C Niemann-Pick disease.
Volume: 17
Issue: 4
Pages: 229-35
Publication
First Author: Sarna JR
Year: 2003
Journal: J Comp Neurol
Title: Patterned Purkinje cell degeneration in mouse models of Niemann-Pick type C disease.
Volume: 456
Issue: 3
Pages: 279-91
Publication
First Author: Miyawaki S
Year: 1983
Journal: J Hered
Title: Time course of hepatic lipids accumulation in a strain of mice with an inherited deficiency of sphingomyelinase.
Volume: 74
Issue: 6
Pages: 465-8
Publication
First Author: Tokoro T
Year: 1991
Journal: Am J Hum Genet
Title: Impaired exogenous cholesterol esterification in Niemann-Pick mouse
Volume: 49
Issue: 4 Suppl
Pages: 107 (Abstr.)
Publication
First Author: Yamada A
Year: 2001
Journal: Brain Dev
Title: Progressive neuronal loss in the ventral posterior lateral and medial nuclei of thalamus in Niemann-Pick disease type C mouse brain.
Volume: 23
Issue: 5
Pages: 288-97
Publication
First Author: Miyawaki S
Year: 1986
Journal: J Hered
Title: A mouse model for Niemann-Pick disease. Influence of genetic background on disease expression in spm/spm mice.
Volume: 77
Issue: 6
Pages: 379-84
Publication
First Author: Erickson RP
Year: 1997
Journal: Mamm Genome
Title: High-resolution mapping of the spm (Niemann-Pick Type C) locus on mouse chromosome 18.
Volume: 8
Issue: 5
Pages: 355-6
Publication  
First Author: Tokoro T
Year: 1996
Journal: Jikeikai Med J
Title: Allelic mutations in two Niemann-Pick disease model mice: SPM (C57BL/KSJ) and NCTR (NCTR-BALB/C).
Volume: 43
Pages: 115-21
Publication
First Author: Vázquez MC
Year: 2011
Journal: PLoS One
Title: Alteration of gene expression profile in Niemann-Pick type C mice correlates with tissue damage and oxidative stress.
Volume: 6
Issue: 12
Pages: e28777
Publication
First Author: Henderson LP
Year: 2000
Journal: J Biol Chem
Title: Embryonic striatal neurons from niemann-pick type C mice exhibit defects in cholesterol metabolism and neurotrophin responsiveness.
Volume: 275
Issue: 26
Pages: 20179-87
Publication
First Author: Houben T
Year: 2020
Journal: J Pathol
Title: Sex-opposed inflammatory effects of 27-hydroxycholesterol are mediated via differences in estrogen signaling.
Volume: 251
Issue: 4
Pages: 429-439
Publication
First Author: Pentchev PG
Year: 1986
Journal: J Biol Chem
Title: The cholesterol storage disorder of the mutant BALB/c mouse. A primary genetic lesion closely linked to defective esterification of exogenously derived cholesterol and its relationship to human type C Niemann-Pick disease.
Volume: 261
Issue: 6
Pages: 2772-7
Publication
First Author: Muralidhar A
Year: 2011
Journal: Mol Genet Metab
Title: Pulmonary function and pathology in hydroxypropyl-beta-cyclodextin-treated and untreated Npc1⁻/⁻ mice.
Volume: 103
Issue: 2
Pages: 142-7
Publication
First Author: Roney JC
Year: 2021
Journal: Dev Cell
Title: Lipid-mediated motor-adaptor sequestration impairs axonal lysosome delivery leading to autophagic stress and dystrophy in Niemann-Pick type C.
Volume: 56
Issue: 10
Pages: 1452-1468.e8
Publication
First Author: Soga M
Year: 2015
Journal: Stem Cells
Title: HPGCD outperforms HPBCD as a potential treatment for Niemann-Pick disease type C during disease modeling with iPS cells.
Volume: 33
Issue: 4
Pages: 1075-88
Publication
First Author: Treiber-Held S
Year: 2003
Journal: J Pathol
Title: Spatial and temporal distribution of intracellular free cholesterol in brains of a Niemann-Pick type C mouse model showing hyperphosphorylated tau protein. Implications for Alzheimer's disease.
Volume: 200
Issue: 1
Pages: 95-103
Publication
First Author: Taylor AM
Year: 2012
Journal: J Lipid Res
Title: Cyclodextrin mediates rapid changes in lipid balance in Npc1-/- mice without carrying cholesterol through the bloodstream.
Volume: 53
Issue: 11
Pages: 2331-42
Publication
First Author: Wu YP
Year: 2005
Journal: Mol Genet Metab
Title: Apoptosis accompanied by up-regulation of TNF-alpha death pathway genes in the brain of Niemann-Pick type C disease.
Volume: 84
Issue: 1
Pages: 9-17
Publication  
First Author: Rabenstein M
Year: 2019
Journal: Int J Mol Sci
Title: Alteration of GABAergic Input Precedes Neurodegeneration of Cerebellar Purkinje Cells of NPC1-Deficient Mice.
Volume: 20
Issue: 24
Publication  
First Author: Shin SD
Year: 2019
Journal: Neurosci Lett
Title: Interferon downstream signaling is activated early in pre-symptomatic Niemann-Pick disease type C.
Volume: 706
Pages: 43-50
Publication
First Author: Zhang M
Year: 2004
Journal: Am J Pathol
Title: Cyclin-dependent kinase inhibitors attenuate protein hyperphosphorylation, cytoskeletal lesion formation, and motor defects in Niemann-Pick Type C mice.
Volume: 165
Issue: 3
Pages: 843-53
Publication
First Author: Higashi Y
Year: 1993
Journal: Acta Neuropathol
Title: Cerebellar degeneration in the Niemann-Pick type C mouse.
Volume: 85
Issue: 2
Pages: 175-84
Publication
First Author: Hovakimyan M
Year: 2013
Journal: PLoS One
Title: Olfactory deficits in Niemann-Pick type C1 (NPC1) disease.
Volume: 8
Issue: 12
Pages: e82216
Publication
First Author: Ohara S
Year: 2004
Journal: Exp Neurol
Title: Axonal dystrophy of dorsal root ganglion sensory neurons in a mouse model of Niemann-Pick disease type C.
Volume: 187
Issue: 2
Pages: 289-98
Publication
First Author: Vainio S
Year: 2005
Journal: Biochem J
Title: Defective insulin receptor activation and altered lipid rafts in Niemann-Pick type C disease hepatocytes.
Volume: 391
Issue: Pt 3
Pages: 465-72
Publication
First Author: Gévry NY
Year: 2004
Journal: Mol Endocrinol
Title: Aberrant intracellular cholesterol transport disrupts pituitary and ovarian function.
Volume: 18
Issue: 7
Pages: 1778-86
Publication
First Author: Xie C
Year: 1999
Journal: Am J Physiol
Title: Cholesterol balance and metabolism in mice with loss of function of Niemann-Pick C protein.
Volume: 276
Issue: 2 Pt 1
Pages: E336-44
Publication
First Author: Pentchev PG
Year: 1984
Journal: J Biol Chem
Title: A genetic storage disorder in BALB/C mice with a metabolic block in esterification of exogenous cholesterol.
Volume: 259
Issue: 9
Pages: 5784-91
Publication
First Author: Karten B
Year: 2002
Journal: J Neurochem
Title: Cholesterol accumulates in cell bodies, but is decreased in distal axons, of Niemann-Pick C1-deficient neurons.
Volume: 83
Issue: 5
Pages: 1154-63
Publication
First Author: Higashi Y
Year: 1995
Journal: Acta Neuropathol
Title: Peripheral nerve pathology in Niemann-Pick type C mouse.
Volume: 90
Issue: 2
Pages: 158-63
Publication
First Author: Li H
Year: 2005
Journal: J Neuropathol Exp Neurol
Title: Molecular, anatomical, and biochemical events associated with neurodegeneration in mice with Niemann-Pick type C disease.
Volume: 64
Issue: 4
Pages: 323-33
Publication
First Author: German DC
Year: 2002
Journal: Neuroscience
Title: Neurodegeneration in the Niemann-Pick C mouse: glial involvement.
Volume: 109
Issue: 3
Pages: 437-50
Publication
First Author: Shio H
Year: 1982
Journal: Am J Pathol
Title: Lysosome lipid storage disorder in NCTR-BALB/c mice. II. Morphologic and cytochemical studies.
Volume: 108
Issue: 2
Pages: 150-9
Publication
First Author: Bhuvaneswaran C
Year: 1982
Journal: Am J Pathol
Title: Lysosome lipid storage disorder in NCTR-BALB/c mice. III. Isolation and analysis of storage inclusions from liver.
Volume: 108
Issue: 2
Pages: 160-70
Publication
First Author: Gévry NY
Year: 2002
Journal: Endocr Res
Title: The role and regulation of the Niemann-Pick C1 gene in adrenal steroidogenesis.
Volume: 28
Issue: 4
Pages: 403-12
Publication
First Author: Klein A
Year: 2011
Journal: Neurobiol Dis
Title: Oxidative stress activates the c-Abl/p73 proapoptotic pathway in Niemann-Pick type C neurons.
Volume: 41
Issue: 1
Pages: 209-18
Publication
First Author: Suresh S
Year: 1998
Journal: J Neurochem
Title: Cellular cholesterol storage in the Niemann-Pick disease type C mouse is associated with increased expression and defective processing of apolipoprotein D.
Volume: 70
Issue: 1
Pages: 242-51
Publication  
First Author: Houben T
Year: 2021
Journal: Front Immunol
Title: Pro-Inflammatory Implications of 2-Hydroxypropyl-β-cyclodextrin Treatment.
Volume: 12
Pages: 716357
Publication
First Author: Yadid G
Year: 1998
Journal: Brain Res
Title: Neurochemical alterations in the cerebellum of a murine model of Niemann-Pick type C disease.
Volume: 799
Issue: 2
Pages: 250-6
Publication
First Author: Goodrum JF
Year: 1997
Journal: J Neurosci Res
Title: Cholesterol reutilization during myelination of regenerating PNS axons is impaired in Niemann-Pick disease type C mice.
Volume: 49
Issue: 3
Pages: 389-92
Publication  
First Author: Yamada Y
Year: 2022
Journal: Biomed Pharmacother
Title: Fine-tuned cholesterol solubilizer, mono-6-O-α-D-maltosyl-γ-cyclodextrin, ameliorates experimental Niemann-Pick disease type C without hearing loss.
Volume: 155
Pages: 113698
Publication  
First Author: Liao G
Year: 2009
Journal: Brain Res
Title: Allopregnanolone treatment delays cholesterol accumulation and reduces autophagic/lysosomal dysfunction and inflammation in Npc1-/- mouse brain.
Volume: 1270
Pages: 140-51
Publication
First Author: Ong QR
Year: 2012
Journal: Biochem Biophys Res Commun
Title: Impaired insulin signaling in an animal model of Niemann-Pick Type C disease.
Volume: 424
Issue: 3
Pages: 482-7
Publication
First Author: Wasser CR
Year: 2007
Journal: J Physiol
Title: Cholesterol-dependent balance between evoked and spontaneous synaptic vesicle recycling.
Volume: 579
Issue: Pt 2
Pages: 413-29
Publication
First Author: Takikita S
Year: 2004
Journal: J Neuropathol Exp Neurol
Title: Perturbed myelination process of premyelinating oligodendrocyte in Niemann-Pick type C mouse.
Volume: 63
Issue: 6
Pages: 660-73
Publication
First Author: Bosch B
Year: 2013
Journal: J Biol Chem
Title: Disruption of multivesicular body vesicles does not affect major histocompatibility complex (MHC) class II-peptide complex formation and antigen presentation by dendritic cells.
Volume: 288
Issue: 34
Pages: 24286-92
Publication  
First Author: Boothe AD
Year: 1977
Journal: Fed Proc
Title: Tissue cholesterol storage disorder in BALB/c mice: histologic findings
Volume: 36
Pages: 1158 (Abstr.)
Publication  
First Author: Morris MD
Year: 1977
Journal: Fed Proc
Title: Tissue cholesterol storage disorder in BALB/c mice
Volume: 36
Pages: 1158 (Abstr.)
Publication
First Author: Weintraub H
Year: 1985
Journal: J Neurochem
Title: Neurological mutation characterized by dysmyelination in NCTR-Balb/C mouse with lysosomal lipid storage disease.
Volume: 45
Issue: 3
Pages: 665-72
Publication
First Author: Patel SC
Year: 1987
Journal: Biochem Biophys Res Commun
Title: Impaired cholesterol esterification in primary brain cultures of the lysosomal cholesterol storage disorder (LCSD) mouse mutant.
Volume: 143
Issue: 1
Pages: 233-40
Publication
First Author: Griffin LD
Year: 2004
Journal: Nat Med
Title: Niemann-Pick type C disease involves disrupted neurosteroidogenesis and responds to allopregnanolone.
Volume: 10
Issue: 7
Pages: 704-11
Publication
First Author: Pentchev PG
Year: 1994
Journal: Biochim Biophys Acta
Title: The Niemann-Pick C lesion and its relationship to the intracellular distribution and utilization of LDL cholesterol.
Volume: 1225
Issue: 3
Pages: 235-43
Publication  
First Author: Gray J
Year: 2022
Journal: EBioMedicine
Title: Heat shock protein amplification improves cerebellar myelination in the Npc1(nih) mouse model.
Volume: 86
Pages: 104374
Publication
First Author: Veyron P
Year: 1996
Journal: Transplantation
Title: Transplantation of fetal liver cells corrects accumulation of lipids in tissues and prevents fatal neuropathy in cholesterol-storage disease BALB/c mice.
Volume: 62
Issue: 8
Pages: 1039-45
Publication
First Author: Bu B
Year: 2002
Journal: J Neurosci
Title: Deregulation of cdk5, hyperphosphorylation, and cytoskeletal pathology in the Niemann-Pick type C murine model.
Volume: 22
Issue: 15
Pages: 6515-25
Publication
First Author: Nunes MJ
Year: 2024
Journal: Biochim Biophys Acta Mol Basis Dis
Title: Cholesterol redistribution triggered by CYP46A1 gene therapy improves major hallmarks of Niemann-Pick type C disease but is not sufficient to halt neurodegeneration.
Volume: 1870
Issue: 3
Pages: 166993
Publication  
First Author: Weintraub H
Year: 1992
Journal: J Cell Sci
Title: Morphometric studies of pancreatic acinar granule formation in NCTR-Balb/c mice.
Volume: 102 ( Pt 1)
Pages: 141-7
Publication
First Author: Carstea ED
Year: 1993
Journal: Proc Natl Acad Sci U S A
Title: Linkage of Niemann-Pick disease type C to human chromosome 18.
Volume: 90
Issue: 5
Pages: 2002-4
Publication
First Author: Kurimasa A
Year: 1993
Journal: Hum Genet
Title: Restoration of the cholesterol metabolism in 3T3 cell lines derived from the sphingomyelinosis mouse (spm/spm) by transfer of a human chromosome 18.
Volume: 92
Issue: 2
Pages: 157-62
Publication
First Author: Gu JZ
Year: 1997
Journal: Proc Natl Acad Sci U S A
Title: Substantial narrowing of the Niemann-Pick C candidate interval by yeast artificial chromosome complementation.
Volume: 94
Issue: 14
Pages: 7378-83
Publication
First Author: Schedin S
Year: 1998
Journal: Biochim Biophys Acta
Title: Alterations in the biosynthesis of cholesterol, dolichol and dolichyl-P in the genetic cholesterol homeostasis disorder, Niemann-Pick type C disease.
Volume: 1394
Issue: 2-3
Pages: 177-86
GXD Expression      
Probe: MGI:1097868
Assay Type: Northern blot
Annotation Date: 2004-07-30
Strength: Present
Sex: Not Specified
Emaps: EMAPS:1610528
Stage: TS28
Assay Id: MGI:3046303
Age: postnatal adult
Specimen Label: Heart
Detected: true
Specimen Num: 1
GXD Expression      
Probe: MGI:1097868
Assay Type: Northern blot
Annotation Date: 2004-07-30
Strength: Present
Sex: Not Specified
Emaps: EMAPS:1689428
Stage: TS28
Assay Id: MGI:3046303
Age: postnatal adult
Specimen Label: Brain
Detected: true
Specimen Num: 2
GXD Expression      
Probe: MGI:1097868
Assay Type: Northern blot
Annotation Date: 2004-07-30
Strength: Present
Sex: Not Specified
Emaps: EMAPS:1876728
Stage: TS28
Assay Id: MGI:3046303
Age: postnatal adult
Specimen Label: Spleen
Detected: true
Specimen Num: 3
GXD Expression      
Probe: MGI:1097868
Assay Type: Northern blot
Annotation Date: 2004-07-30
Strength: Present
Sex: Not Specified
Emaps: EMAPS:1672828
Stage: TS28
Assay Id: MGI:3046303
Age: postnatal adult
Specimen Label: Lung
Detected: true
Specimen Num: 4
GXD Expression      
Probe: MGI:1097868
Assay Type: Northern blot
Annotation Date: 2004-07-30
Strength: Present
Sex: Not Specified
Emaps: EMAPS:1684628
Stage: TS28
Assay Id: MGI:3046303
Age: postnatal adult
Specimen Label: Liver
Detected: true
Specimen Num: 5