Selective neurodegeneration, without neurofibrillary tangles, in a mouse model of Niemann-Pick C disease.
about
Tau deletion exacerbates the phenotype of Niemann-Pick type C mice and implicates autophagy in pathogenesis.Cell-autonomous death of cerebellar purkinje neurons with autophagy in Niemann-Pick type C diseaseAutophagy in the physiology and pathology of the central nervous systemHearing loss is an early consequence of Npc1 gene deletion in the mouse model of Niemann-Pick disease, type C.Niemann-Pick C disease and mobilization of lysosomal cholesterol by cyclodextrinAltered levels and distribution of amyloid precursor protein and its processing enzymes in Niemann-Pick type C1-deficient mouse brainsSize and shape of the corpus callosum in adult Niemann-Pick type C reflects state and trait illness variables.Alterations in gene expression in mutant amyloid precursor protein transgenic mice lacking Niemann-Pick type C1 proteinAnatomically defined neuron-based rescue of neurodegenerative Niemann-Pick type C disorder.Altered vitamin E status in Niemann-Pick type C disease.Niemann-Pick Type C disease and Alzheimer's disease: the APP-endosome connection fattens up.Cyclin-dependent kinase inhibitors attenuate protein hyperphosphorylation, cytoskeletal lesion formation, and motor defects in Niemann-Pick Type C mice.Neuronal loss of Drosophila NPC1a causes cholesterol aggregation and age-progressive neurodegeneration.Loss of Cathepsin B and L Leads to Lysosomal Dysfunction, NPC-Like Cholesterol Sequestration and Accumulation of the Key Alzheimer's ProteinsAberrant lysosomal carbohydrate storage accompanies endocytic defects and neurodegeneration in Drosophila benchwarmer.Quantitative magnetic resonance imaging of brain atrophy in a mouse model of Niemann-Pick type C disease.Lipid imbalance in the neurological disorder, Niemann-Pick C disease.Necroptosis in Niemann-Pick disease, type C1: a potential therapeutic target.A human neuronal model of Niemann Pick C disease developed from stem cells isolated from patient's skinEarly glial activation, synaptic changes and axonal pathology in the thalamocortical system of Niemann-Pick type C1 mice.Dysfunctional cell-cell signaling in the neurovascular unit as a paradigm for central nervous system disease.Astrocyte-only Npc1 reduces neuronal cholesterol and triples life span of Npc1-/- mice.Defective cholesterol trafficking in Niemann-Pick C-deficient cells.Current controversies in Niemann-Pick C1 disease: steroids or gangliosides; neurons or neurons and glia.Activation of PKC triggers rescue of NPC1 patient specific iPSC derived glial cells from gliosis.Longitudinal changes in cerebellar and subcortical volumes in adult-onset Niemann-Pick disease type C patients treated with miglustat.Increased Regenerative Capacity of the Olfactory Epithelium in Niemann-Pick Disease Type C1.Increased activity and altered subcellular distribution of lysosomal enzymes determine neuronal vulnerability in Niemann-Pick type C1-deficient mice.Unesterified cholesterol accumulation in late endosomes/lysosomes causes neurodegeneration and is prevented by driving cholesterol export from this compartment.Role of cathepsin D in U18666A-induced neuronal cell death: potential implication in Niemann-Pick type C disease pathogenesis.Studies on neuronal death in the mouse model of Niemann-Pick C disease.Quantitation of two pathways for cholesterol excretion from the brain in normal mice and mice with neurodegeneration.Niemann-Pick type C disease involves disrupted neurosteroidogenesis and responds to allopregnanolone.Subcortical volumetric reductions in adult Niemann-Pick disease type C: a cross-sectional study.Cholesterol substrate pools and steroid hormone levels are normal in the face of mutational inactivation of NPC1 protein.Receptor-mediated and bulk-phase endocytosis cause macrophage and cholesterol accumulation in Niemann-Pick C disease.Reduced cerebellar neurodegeneration after combined therapy with cyclodextrin/allopregnanolone and miglustat in NPC1: a mouse model of Niemann-Pick type C1 disease.Decreased purinergic inhibition of synaptic activity in a mouse model of Niemann-Pick disease type C.Miglustat in Niemann-Pick disease type C patients: a review
P2860
Q24328779-024F5CFF-A3DF-465E-A541-1797390B1C65Q24811579-D33B09FC-B4FD-4628-9C09-4B1E89B032B9Q27000701-39D3AE85-8582-4DFB-8E70-D75C14A970DBQ30405777-A5BEE9D7-441E-4F96-98F7-93BF903885EBQ33945068-015709EF-BE39-4C6B-ABC7-47B0C1AA3F5EQ34039082-B9AD82F4-FABA-4628-AD6F-A2486423F2FCQ34186279-AA908220-E8E1-4807-BB8B-C0689EDCE77CQ34575511-86A32B46-9037-4AE9-AA4A-5C2666E0A803Q34766860-3DBC8776-E4FD-4CAB-8D2A-930FBD48E580Q35067280-F5018AF6-7E62-4731-AF7D-340C1C758F69Q35094638-EB4D1C15-D561-4E6A-8CF0-A96A4762691AQ35103069-55C1EE4B-C795-4291-801D-C9EE04020D2DQ35835807-E6DD521B-AE36-41BF-9515-B1F27AE2863AQ36208265-A54E9810-07B0-47F3-88EB-1CC1DB7DCEC5Q36320691-B4B55909-64C8-4827-AD70-70E90B895368Q36380510-3C8CD3D0-7EA6-4DCE-AF2F-5EEFDCC7D09AQ36517103-6870B68E-4774-4FAA-A2BA-DB9D5E8256CAQ36774925-FC1BBF60-C18F-4557-AF01-DFC7DF03C7AEQ36828054-CF279757-1A19-4C10-96B9-8C627B278355Q36852911-8A7B5C27-6409-4441-8520-363FAA076860Q37017486-2342C08F-DD7E-48A2-B074-110C83DEAED7Q37080277-222D041C-58AB-4D20-B17B-D709EFA80D63Q37736657-5BFFDE50-468D-4CE4-9821-E4DF5283C9A6Q38071990-9DA36AC1-C825-459A-9038-C7D03EAA2DDFQ38607944-FF683256-CCD7-4D87-A842-46F415A138B7Q40818505-4A36F9A7-9E6E-4AB4-A026-5A1C7419BB5CQ41953682-75F82935-F7AD-422B-892A-A7C606FF2CAFQ42139404-CE0074FF-6E6E-489E-AEDC-80D23B381639Q42140225-2A5600B0-9FCC-40D2-AA2E-2FDB6E2A5103Q43118769-50D94F7D-E6A8-4623-8A2B-01DB4F257842Q44059850-6F07BC0F-BA9F-4E36-90C2-828BA35E1B9FQ44480882-22737FD9-3116-4D85-8904-6342E5EA6D12Q44945736-01044722-5C9F-4E31-83B1-799C7C395604Q44998717-29C68780-547C-4D2E-9B62-14E94CD4CB81Q46930679-E3971CCD-7FE5-4BDC-B581-D75D4FE9FD23Q47598679-1528BCA5-41EB-46A6-8FF1-B6D3385EE416Q47789865-4179AA51-7537-4827-A7CD-CDB80398AB21Q48342013-8EE09C14-38DA-4AD0-B717-57942EBCBC9DQ58778074-8029EAEC-EEC1-40A9-B727-D31BCD399CFB
P2860
Selective neurodegeneration, without neurofibrillary tangles, in a mouse model of Niemann-Pick C disease.
description
2001 nî lūn-bûn
@nan
2001年の論文
@ja
2001年学术文章
@wuu
2001年学术文章
@zh-cn
2001年学术文章
@zh-hans
2001年学术文章
@zh-my
2001年学术文章
@zh-sg
2001年學術文章
@yue
2001年學術文章
@zh
2001年學術文章
@zh-hant
name
Selective neurodegeneration, w ...... del of Niemann-Pick C disease.
@ast
Selective neurodegeneration, w ...... del of Niemann-Pick C disease.
@en
type
label
Selective neurodegeneration, w ...... del of Niemann-Pick C disease.
@ast
Selective neurodegeneration, w ...... del of Niemann-Pick C disease.
@en
prefLabel
Selective neurodegeneration, w ...... del of Niemann-Pick C disease.
@ast
Selective neurodegeneration, w ...... del of Niemann-Pick C disease.
@en
P2093
P2860
P356
P1476
Selective neurodegeneration, w ...... del of Niemann-Pick C disease.
@en
P2093
D C German
E M Quintero
J M Dietschy
P2860
P304
P356
10.1002/CNE.1149
P407
P577
2001-05-01T00:00:00Z