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Search results 101 to 200 out of 560 for Npc1

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Type Details Score
Publication
15652985 | J:105214
First Author: Deisz RA
Year: 2005
Journal: Neuroscience
Title: Pathological cholesterol metabolism fails to modify electrophysiological properties of afflicted neurones in Niemann-Pick disease type C.
Volume: 130
Issue: 4
Pages: 867-73
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Publication
19884502 | J:154766
First Author: Abi-Mosleh L
Year: 2009
Journal: Proc Natl Acad Sci U S A
Title: Cyclodextrin overcomes deficient lysosome-to-endoplasmic reticulum transport of cholesterol in Niemann-Pick type C cells.
Volume: 106
Issue: 46
Pages: 19316-21
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Publication
27798114 | J:241449
First Author: Chandler RJ
Year: 2017
Journal: Hum Mol Genet
Title: Systemic AAV9 gene therapy improves the lifespan of mice with Niemann-Pick disease, type C1.
Volume: 26
Issue: 1
Pages: 52-64
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Publication
19893049 | J:155318
First Author: Amritraj A
Year: 2009
Journal: Am J Pathol
Title: Increased activity and altered subcellular distribution of lysosomal enzymes determine neuronal vulnerability in Niemann-Pick type C1-deficient mice.
Volume: 175
Issue: 6
Pages: 2540-56
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Publication
32770132 | J:298953
First Author: Jiang D
Year: 2020
Journal: Sci Rep
Title: Plasmid DNA gene therapy of the Niemann-Pick C1 mouse with transferrin receptor-targeted Trojan horse liposomes.
Volume: 10
Issue: 1
Pages: 13334
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Publication
17107950 | J:118542
First Author: Kulinski A
Year: 2007
Journal: J Biol Chem
Title: Lipid homeostasis and lipoprotein secretion in Niemann-Pick C1-deficient hepatocytes.
Volume: 282
Issue: 3
Pages: 1627-37
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Publication
17220530 | J:121676
First Author: Beltroy EP
Year: 2007
Journal: J Lipid Res
Title: Lysosomal unesterified cholesterol content correlates with liver cell death in murine Niemann-Pick type C disease.
Volume: 48
Issue: 4
Pages: 869-81
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Publication
28487438 | J:245064
First Author: Castillo JJ
Year: 2017
Journal: Am J Physiol Endocrinol Metab
Title: The Niemann-Pick C1 gene interacts with a high-fat diet to promote weight gain through differential regulation of central energy metabolism pathways.
Volume: 313
Issue: 2
Pages: E183-E194
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Publication
22198570 | J:182040
First Author: Pressey SN
Year: 2012
Journal: Neurobiol Dis
Title: Early glial activation, synaptic changes and axonal pathology in the thalamocortical system of Niemann-Pick type C1 mice.
Volume: 45
Issue: 3
Pages: 1086-100
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Publication
12611909 | J:83463
First Author: Reid PC
Year: 2003
Journal: J Lipid Res
Title: Trafficking defects in endogenously synthesized cholesterol in fibroblasts, macrophages, hepatocytes, and glial cells from Niemann-Pick type C1 mice.
Volume: 44
Issue: 5
Pages: 1010-9
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Publication
19056848 | J:144240
First Author: Kaptzan T
Year: 2009
Journal: Am J Pathol
Title: Development of a Rab9 transgenic mouse and its ability to increase the lifespan of a murine model of Niemann-Pick type C disease.
Volume: 174
Issue: 1
Pages: 14-20
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Publication
33503462 | J:343040
 
First Author: Ashikawa H
Year: 2021
Journal: Eur J Pharmacol
Title: Beneficial effects of primidone in Niemann-Pick disease type C (NPC)-model cells and mice: Reduction of unesterified cholesterol levels in cells and extension of lifespan in mice.
Volume: 896
Pages: 173907
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Publication
20489167 | J:179556
First Author: Uronen RL
Year: 2010
Journal: Arterioscler Thromb Vasc Biol
Title: Niemann-Pick C1 modulates hepatic triglyceride metabolism and its genetic variation contributes to serum triglyceride levels.
Volume: 30
Issue: 8
Pages: 1614-20
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Publication
30666257 | J:284453
 
First Author: Houben T
Year: 2018
Journal: Front Immunol
Title: Pneumococcal Immunization Reduces Neurological and Hepatic Symptoms in a Mouse Model for Niemann-Pick Type C1 Disease.
Volume: 9
Pages: 3089
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Publication
22020183 | J:179071
First Author: Jelinek DA
Year: 2012
Journal: Gene
Title: Genetic variation in the mouse model of Niemann Pick C1 affects female, as well as male, adiposity, and hepatic bile transporters but has indeterminate effects on caveolae.
Volume: 491
Issue: 2
Pages: 128-34
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Publication
24235134 | J:208090
First Author: Speak AO
Year: 2014
Journal: Blood
Title: Altered distribution and function of natural killer cells in murine and human Niemann-Pick disease type C1.
Volume: 123
Issue: 1
Pages: 51-60
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Publication
30266834 | J:267311
First Author: Tobias F
Year: 2018
Journal: J Lipid Res
Title: Mass spectrometry imaging of lipids: untargeted consensus spectra reveal spatial distributions in Niemann-Pick disease type C1.
Volume: 59
Issue: 12
Pages: 2446-2455
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Publication
12458210 | J:81676
First Author: Karten B
Year: 2003
Journal: J Biol Chem
Title: Trafficking of cholesterol from cell bodies to distal axons in Niemann Pick C1-deficient neurons.
Volume: 278
Issue: 6
Pages: 4168-75
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Publication
21704157 | J:177615
First Author: Peake KB
Year: 2011
Journal: Biochim Biophys Acta
Title: Niemann-Pick Type C1 deficiency in microglia does not cause neuron death in vitro.
Volume: 1812
Issue: 9
Pages: 1121-9
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Publication
29878115 | J:264504
First Author: Hughes MP
Year: 2018
Journal: Hum Mol Genet
Title: AAV9 intracerebroventricular gene therapy improves lifespan, locomotor function and pathology in a mouse model of Niemann-Pick type C1 disease.
Volume: 27
Issue: 17
Pages: 3079-3098
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Publication
18591368 | J:140248
First Author: Alvarez AR
Year: 2008
Journal: FASEB J
Title: Imatinib therapy blocks cerebellar apoptosis and improves neurological symptoms in a mouse model of Niemann-Pick type C disease.
Volume: 22
Issue: 10
Pages: 3617-27
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Publication
23881911 | J:202626
First Author: Fan M
Year: 2013
Journal: J Lipid Res
Title: Identification of Niemann-Pick C1 disease biomarkers through sphingolipid profiling.
Volume: 54
Issue: 10
Pages: 2800-14
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Publication
22869680 | J:188345
First Author: Maulik M
Year: 2012
Journal: Hum Mol Genet
Title: Mutant human APP exacerbates pathology in a mouse model of NPC and its reversal by a β-cyclodextrin.
Volume: 21
Issue: 22
Pages: 4857-75
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Publication
16850391 | J:119302
First Author: Fan J
Year: 2006
Journal: Mol Reprod Dev
Title: Sperm defects in mice lacking a functional Niemann-Pick C1 protein.
Volume: 73
Issue: 10
Pages: 1284-91
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Publication
12297829 | J:106027
First Author: Amigo L
Year: 2002
Journal: Hepatology
Title: Relevance of Niemann-Pick type C1 protein expression in controlling plasma cholesterol and biliary lipid secretion in mice.
Volume: 36
Issue: 4 Pt 1
Pages: 819-28
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Publication
16940355 | J:113746
First Author: Langmade SJ
Year: 2006
Journal: Proc Natl Acad Sci U S A
Title: Pregnane X receptor (PXR) activation: a mechanism for neuroprotection in a mouse model of Niemann-Pick C disease.
Volume: 103
Issue: 37
Pages: 13807-12
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Publication
27083515 | J:233698
First Author: Kennedy BE
Year: 2016
Journal: Am J Pathol
Title: Presymptomatic Alterations in Amino Acid Metabolism and DNA Methylation in the Cerebellum of a Murine Model of Niemann-Pick Type C Disease.
Volume: 186
Issue: 6
Pages: 1582-97
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Publication
11152466 | J:69686
First Author: Sawamura N
Year: 2001
Journal: J Biol Chem
Title: Site-specific phosphorylation of tau accompanied by activation of mitogen-activated protein kinase (MAPK) in brains of Niemann-Pick type C mice.
Volume: 276
Issue: 13
Pages: 10314-9
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Publication
25009206 | J:213845
First Author: Akpovi CD
Year: 2014
Journal: Biol Reprod
Title: Dysregulation of testicular cholesterol metabolism following spontaneous mutation of the niemann-pick c1 gene in mice.
Volume: 91
Issue: 2
Pages: 42
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Publication
20456004 | J:161819
First Author: Hawes CM
Year: 2010
Journal: J Neurochem
Title: Pre-synaptic defects of NPC1-deficient hippocampal neurons are not directly related to plasma membrane cholesterol.
Volume: 114
Issue: 1
Pages: 311-22
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Publication
14703504 | J:88630
First Author: Reid PC
Year: 2004
Journal: J Lipid Res
Title: A novel cholesterol stain reveals early neuronal cholesterol accumulation in the Niemann-Pick type C1 mouse brain.
Volume: 45
Issue: 3
Pages: 582-91
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Publication
23666527 | J:199190
First Author: Fu R
Year: 2013
Journal: Hum Mol Genet
Title: Efficacy of N-acetylcysteine in phenotypic suppression of mouse models of Niemann-Pick disease, type C1.
Volume: 22
Issue: 17
Pages: 3508-23
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Publication
17624314 | J:123024
First Author: Kim SJ
Year: 2007
Journal: Biochem Biophys Res Commun
Title: Defective cholesterol traffic and neuronal differentiation in neural stem cells of Niemann-Pick type C disease improved by valproic acid, a histone deacetylase inhibitor.
Volume: 360
Issue: 3
Pages: 593-9
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Publication
22120593 | J:180321
First Author: Yévenes LF
Year: 2012
Journal: Biochim Biophys Acta
Title: Lysosomal vitamin E accumulation in Niemann-Pick type C disease.
Volume: 1822
Issue: 2
Pages: 150-60
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Publication
18190929 | J:139928
First Author: Luan Z
Year: 2008
Journal: J Neurol Sci
Title: Brainstem neuropathology in a mouse model of Niemann-Pick disease type C.
Volume: 268
Issue: 1-2
Pages: 108-16
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Publication
21048217 | J:168029
First Author: Porter FD
Year: 2010
Journal: Sci Transl Med
Title: Cholesterol oxidation products are sensitive and specific blood-based biomarkers for Niemann-Pick C1 disease.
Volume: 2
Issue: 56
Pages: 56ra81
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Publication
21866103 | J:176235
First Author: Carette JE
Year: 2011
Journal: Nature
Title: Ebola virus entry requires the cholesterol transporter Niemann-Pick C1.
Volume: 477
Issue: 7364
Pages: 340-3
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Publication
25132229 | J:218464
First Author: Seo Y
Year: 2014
Journal: Biochim Biophys Acta
Title: Excessive microglial activation aggravates olfactory dysfunction by impeding the survival of newborn neurons in the olfactory bulb of Niemann-Pick disease type C1 mice.
Volume: 1842
Issue: 11
Pages: 2193-203
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Publication
24964810 | J:214969
First Author: Tortelli B
Year: 2014
Journal: Hum Mol Genet
Title: Cholesterol homeostatic responses provide biomarkers for monitoring treatment for the neurodegenerative disease Niemann-Pick C1 (NPC1).
Volume: 23
Issue: 22
Pages: 6022-33
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Publication
20607864 | J:168042
First Author: Kodam A
Year: 2010
Journal: Glia
Title: Altered levels and distribution of amyloid precursor protein and its processing enzymes in Niemann-Pick type C1-deficient mouse brains.
Volume: 58
Issue: 11
Pages: 1267-81
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Publication
24969023 | J:215476
 
First Author: Nusca S
Year: 2014
Journal: Neurobiol Dis
Title: A marked paucity of granule cells in the developing cerebellum of the Npc1(-/-) mouse is corrected by a single injection of hydroxypropyl-β-cyclodextrin.
Volume: 70
Pages: 117-26
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Publication
18953351 | J:144082
First Author: Lloyd-Evans E
Year: 2008
Journal: Nat Med
Title: Niemann-Pick disease type C1 is a sphingosine storage disease that causes deregulation of lysosomal calcium.
Volume: 14
Issue: 11
Pages: 1247-55
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Publication
25472750 | J:280576
 
First Author: Yan X
Year: 2014
Journal: BMC Neurosci
Title: Defects in the retina of Niemann-pick type C 1 mutant mice.
Volume: 15
Pages: 126
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Publication
23769925 | J:200780
First Author: Jelinek D
Year: 2013
Journal: Gene
Title: The C57BL/6J Niemann-Pick C1 mouse model with decreased gene dosage has impaired glucose tolerance independent of body weight.
Volume: 527
Issue: 1
Pages: 65-70
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Publication
25319340 | J:221070
First Author: Marschalek N
Year: 2015
Journal: J Neurochem
Title: Geranylgeranyl pyrophosphate is crucial for neuronal survival but has no special role in Purkinje cell degeneration in Niemann Pick type C1 disease.
Volume: 133
Issue: 1
Pages: 153-61
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Publication
21866101 | J:219073
First Author: Côté M
Year: 2011
Journal: Nature
Title: Small molecule inhibitors reveal Niemann-Pick C1 is essential for Ebola virus infection.
Volume: 477
Issue: 7364
Pages: 344-8
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Publication
11525744 | J:71464
First Author: Zervas M
Year: 2001
Journal: Curr Biol
Title: Critical role for glycosphingolipids in Niemann-Pick disease type C.
Volume: 11
Issue: 16
Pages: 1283-7
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10482798 | J:56837
First Author: Falk T
Year: 1999
Journal: Brain Res
Title: Expression of Niemann-Pick type C transcript in rodent cerebellum in vivo and in vitro.
Volume: 839
Issue: 1
Pages: 49-57
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15930512 | J:100479
First Author: Garver WS
Year: 2005
Journal: J Lipid Res
Title: Niemann-Pick C1 expression is not regulated by the amount of cholesterol flowing through cells in the mouse.
Volume: 46
Issue: 8
Pages: 1745-54
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Publication
17895371 | J:128336
First Author: Berger AC
Year: 2007
Journal: J Cell Sci
Title: The subcellular localization of the Niemann-Pick Type C proteins depends on the adaptor complex AP-3.
Volume: 120
Issue: Pt 20
Pages: 3640-52
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Publication
24412309 | J:211612
 
First Author: Meske V
Year: 2014
Journal: Neurobiol Dis
Title: The autophagic defect in Niemann-Pick disease type C neurons differs from somatic cells and reduces neuronal viability.
Volume: 64
Pages: 88-97
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Publication
31004654 | J:289724
 
First Author: Meske V
Year: 2019
Journal: Neuropharmacology
Title: NPC1-deficient neurons are selectively vulnerable for statin treatment.
Volume: 151
Pages: 159-170
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Publication
18483620 | J:305748
First Author: Zhang JR
Year: 2008
Journal: J Clin Invest
Title: Niemann-Pick C1 protects against atherosclerosis in mice via regulation of macrophage intracellular cholesterol trafficking.
Volume: 118
Issue: 6
Pages: 2281-90
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Publication
16785493 | J:134414
First Author: Sagiv Y
Year: 2006
Journal: J Immunol
Title: Cutting edge: impaired glycosphingolipid trafficking and NKT cell development in mice lacking Niemann-Pick type C1 protein.
Volume: 177
Issue: 1
Pages: 26-30
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Publication
24290752 | J:206841
First Author: Sarkar S
Year: 2013
Journal: Cell Rep
Title: Impaired autophagy in the lipid-storage disorder Niemann-Pick type C1 disease.
Volume: 5
Issue: 5
Pages: 1302-15
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Publication
15355983 | J:95002
First Author: Mutka AL
Year: 2004
Journal: J Biol Chem
Title: Secretion of sterols and the NPC2 protein from primary astrocytes.
Volume: 279
Issue: 47
Pages: 48654-62
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Publication
27019000 | J:257385
First Author: Nicoli ER
Year: 2016
Journal: PLoS One
Title: Defective Cytochrome P450-Catalysed Drug Metabolism in Niemann-Pick Type C Disease.
Volume: 11
Issue: 3
Pages: e0152007
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Publication
14769381 | J:87264
First Author: Baudry M
Year: 2003
Journal: Exp Neurol
Title: Postnatal development of inflammation in a murine model of Niemann-Pick type C disease: immunohistochemical observations of microglia and astroglia.
Volume: 184
Issue: 2
Pages: 887-903
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Publication
19171898 | J:146298
First Author: Liu B
Year: 2009
Journal: Proc Natl Acad Sci U S A
Title: Reversal of defective lysosomal transport in NPC disease ameliorates liver dysfunction and neurodegeneration in the npc1-/- mouse.
Volume: 106
Issue: 7
Pages: 2377-82
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Publication
24631719 | J:213658
 
First Author: Williams IM
Year: 2014
Journal: Neurobiol Dis
Title: Improved neuroprotection using miglustat, curcumin and ibuprofen as a triple combination therapy in Niemann-Pick disease type C1 mice.
Volume: 67
Pages: 9-17
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Publication
23382922 | J:195915
First Author: Maulik M
Year: 2013
Journal: PLoS One
Title: Alterations in gene expression in mutant amyloid precursor protein transgenic mice lacking Niemann-Pick type C1 protein.
Volume: 8
Issue: 1
Pages: e54605
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Publication
24367541 | J:211133
First Author: Kennedy BE
Year: 2013
Journal: PLoS One
Title: Pre-symptomatic activation of antioxidant responses and alterations in glucose and pyruvate metabolism in Niemann-Pick Type C1-deficient murine brain.
Volume: 8
Issue: 12
Pages: e82685
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Publication
21575932 | J:172649
 
First Author: D'Arcangelo G
Year: 2011
Journal: Brain Res
Title: Glutamatergic neurotransmission in a mouse model of Niemann-Pick type C disease.
Volume: 1396
Pages: 11-9
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Publication
18216017 | J:135164
First Author: Gelsthorpe ME
Year: 2008
Journal: J Biol Chem
Title: Niemann-Pick type C1 I1061T mutant encodes a functional protein that is selected for endoplasmic reticulum-associated degradation due to protein misfolding.
Volume: 283
Issue: 13
Pages: 8229-36
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Publication
35563467 | J:327515
 
First Author: Cawley NX
Year: 2022
Journal: Int J Mol Sci
Title: Complex N-Linked Glycosylation: A Potential Modifier of Niemann-Pick Disease, Type C1 Pathology.
Volume: 23
Issue: 9
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Publication
15862536 | J:98024
First Author: Kim MJ
Year: 2005
Journal: Brain Res
Title: Substance P immunoreactive cell reductions in cerebral cortex of Niemann-Pick disease type C mouse.
Volume: 1043
Issue: 1-2
Pages: 218-24
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Publication
33345999 | J:304080
First Author: Meneses-Salas E
Year: 2021
Journal: Am J Pathol
Title: Lack of Annexin A6 Exacerbates Liver Dysfunction and Reduces Lifespan of Niemann-Pick Type C Protein-Deficient Mice.
Volume: 191
Issue: 3
Pages: 475-486
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11394880 | J:69915
First Author: Erickson RP
Year: 2001
Journal: Biochem Biophys Res Commun
Title: Cholesterol signaling at the endoplasmic reticulum occurs in npc1(-/-) but not in npc1(-/-), LDLR(-/-) mice.
Volume: 284
Issue: 2
Pages: 326-30
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Publication
29617956 | J:262961
First Author: Cougnoux A
Year: 2018
Journal: Hum Mol Genet
Title: Microglia activation in Niemann-Pick disease, type C1 is amendable to therapeutic intervention.
Volume: 27
Issue: 12
Pages: 2076-2089
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Publication
22065762 | J:180176
First Author: Deffieu MS
Year: 2011
Journal: Proc Natl Acad Sci U S A
Title: Niemann-Pick type C 1 function requires lumenal domain residues that mediate cholesterol-dependent NPC2 binding.
Volume: 108
Issue: 47
Pages: 18932-6
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Publication
32244519 | J:301023
 
First Author: Cawley NX
Year: 2020
Journal: Int J Mol Sci
Title: Evaluation of the Potential Role of Proprotein Convertase Subtilisin/Kexin Type 9 (PCSK9) in Niemann-Pick Disease, Type C1.
Volume: 21
Issue: 7
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12906793 | J:95799
First Author: Gondré-Lewis MC
Year: 2003
Journal: Curr Biol
Title: Cholesterol accumulation in NPC1-deficient neurons is ganglioside dependent.
Volume: 13
Issue: 15
Pages: 1324-9
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10767333 | J:61777
First Author: Liu Y
Year: 2000
Journal: Hum Mol Genet
Title: Alleviation of neuronal ganglioside storage does not improve the clinical course of the Niemann-Pick C disease mouse.
Volume: 9
Issue: 7
Pages: 1087-92
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Publication
26908626 | J:231438
First Author: Chung C
Year: 2016
Journal: Hum Mol Genet
Title: Genetic and pharmacological evidence implicates cathepsins in Niemann-Pick C cerebellar degeneration.
Volume: 25
Issue: 7
Pages: 1434-46
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Publication
24747682 | J:212556
First Author: Mundy DI
Year: 2014
Journal: Biochim Biophys Acta
Title: Impact of the loss of caveolin-1 on lung mass and cholesterol metabolism in mice with and without the lysosomal cholesterol transporter, Niemann-Pick type C1.
Volume: 1841
Issue: 7
Pages: 995-1002
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Publication
19074461 | J:145003
First Author: Pacheco CD
Year: 2009
Journal: Hum Mol Genet
Title: Tau deletion exacerbates the phenotype of Niemann-Pick type C mice and implicates autophagy in pathogenesis.
Volume: 18
Issue: 5
Pages: 956-65
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Publication
17476031 | J:123778
First Author: Liu B
Year: 2007
Journal: J Lipid Res
Title: Receptor-mediated and bulk-phase endocytosis cause macrophage and cholesterol accumulation in Niemann-Pick C disease.
Volume: 48
Issue: 8
Pages: 1710-23
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Publication
21550990 | J:174801
First Author: Ulatowski L
Year: 2011
Journal: J Lipid Res
Title: Altered vitamin E status in Niemann-Pick type C disease.
Volume: 52
Issue: 7
Pages: 1400-10
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Publication
21887293 | J:176152
First Author: Dixit SS
Year: 2011
Journal: PLoS One
Title: Loss of Niemann-Pick C1 or C2 protein results in similar biochemical changes suggesting that these proteins function in a common lysosomal pathway.
Volume: 6
Issue: 8
Pages: e23677
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Publication
23948640 | J:207440
 
First Author: Hovakimyan M
Year: 2013
Journal: Neuroscience
Title: Combined therapy with cyclodextrin/allopregnanolone and miglustat improves motor but not cognitive functions in Niemann-Pick Type C1 mice.
Volume: 252
Pages: 201-11
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Publication
17984379 | J:142987
First Author: Welch CL
Year: 2007
Journal: Circulation
Title: Spontaneous atherothrombosis and medial degradation in Apoe-/-, Npc1-/- mice.
Volume: 116
Issue: 21
Pages: 2444-52
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Publication
28379564 | J:242832
First Author: Canterini S
Year: 2017
Journal: Hum Mol Genet
Title: Shortened primary cilium length and dysregulated Sonic hedgehog signaling in Niemann-Pick C1 disease.
Volume: 26
Issue: 12
Pages: 2277-2289
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Publication
11984826 | J:76395
First Author: Erickson RP
Year: 2002
Journal: Mol Reprod Dev
Title: mdr1a deficiency corrects sterility in Niemann-Pick C1 protein deficient female mice.
Volume: 62
Issue: 2
Pages: 167-73
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Publication
21697390 | J:173597
First Author: Aqul A
Year: 2011
Journal: J Neurosci
Title: Unesterified cholesterol accumulation in late endosomes/lysosomes causes neurodegeneration and is prevented by driving cholesterol export from this compartment.
Volume: 31
Issue: 25
Pages: 9404-13
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Publication
17468177 | J:125097
First Author: Pacheco CD
Year: 2007
Journal: Hum Mol Genet
Title: Autophagy in Niemann-Pick C disease is dependent upon Beclin-1 and responsive to lipid trafficking defects.
Volume: 16
Issue: 12
Pages: 1495-503
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Publication
23977027 | J:206348
First Author: Sáez PJ
Year: 2013
Journal: PLoS One
Title: Disruption in connexin-based communication is associated with intracellular Ca²⁺ signal alterations in astrocytes from Niemann-Pick type C mice.
Volume: 8
Issue: 8
Pages: e71361
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Publication
37369603 | J:337465
 
First Author: Yasuda T
Year: 2023
Journal: Life Sci Alliance
Title: Peripheral immune system modulates Purkinje cell degeneration in Niemann-Pick disease type C1.
Volume: 6
Issue: 9
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Publication
27687148 | J:236227
First Author: Seo Y
Year: 2016
Journal: Glia
Title: Cathepsin S contributes to microglia-mediated olfactory dysfunction through the regulation of Cx3cl1-Cx3cr1 axis in a Niemann-Pick disease type C1 model.
Volume: 64
Issue: 12
Pages: 2291-2305
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Publication
23843985 | J:204311
First Author: Roszell BR
Year: 2013
Journal: PLoS One
Title: Pulmonary abnormalities in animal models due to Niemann-Pick type C1 (NPC1) or C2 (NPC2) disease.
Volume: 8
Issue: 7
Pages: e67084
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Publication
24076310 | J:210049
First Author: Ramirez CM
Year: 2014
Journal: Biochim Biophys Acta
Title: Ontogenic changes in lung cholesterol metabolism, lipid content, and histology in mice with Niemann-Pick type C disease.
Volume: 1841
Issue: 1
Pages: 54-61
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Publication
27466344 | J:234624
First Author: Demais V
Year: 2016
Journal: J Neurosci
Title: Reversal of Pathologic Lipid Accumulation in NPC1-Deficient Neurons by Drug-Promoted Release of LAMP1-Coated Lamellar Inclusions.
Volume: 36
Issue: 30
Pages: 8012-25
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Publication
21708114 | J:174399
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
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Publication
20864542 | J:167334
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
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Publication
31999726 | J:285529
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
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Publication
20386595 | J:160166
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
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Publication
17314284 | J:118352
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
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Publication
17553802 | J:124579
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
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Publication
29979789 | J:263821
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
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Gene
108711582 | npc1l1.L
Type: gene
Organism: frog, African clawed
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Gene
108712289 | npc1l1.S
Type: gene
Organism: frog, African clawed
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MouseMine is a collaboration between MGI at The Jackson Laboratory and the InterMine project at the Cambridge Systems Biology Centre. MouseMine is funded through a grant from the NIH/NHGRI.The Jackson Laboratory makes no representation about the suitability or accuracy of this software or data for any purpose, and makes no warranties, either express or implied, including merchantability and fitness for a particular purpose or that the use of this software or data will not infringe any third party patents, copyrights, trademarks, or other rights. the software and data are provided "as is". This software and data are provided to enhance knowledge and encourage progress in the scientific community and are to be used only for research and educational purposes. Any reproduction or use for commercial purpose is prohibited without the prior express written permission of the Jackson Laboratory.

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