Increased activity and altered subcellular distribution of lysosomal enzymes determine neuronal vulnerability in Niemann-Pick type C1-deficient mice.
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Guidelines for the use and interpretation of assays for monitoring autophagyCathepsin B is a New Drug Target for Traumatic Brain Injury Therapeutics: Evidence for E64d as a Promising Lead Drug CandidateLysosomal storage diseases and the heat shock response: convergences and therapeutic opportunitiesOlfactory deficits in Niemann-Pick type C1 (NPC1) diseaseLoss of Niemann-Pick C1 or C2 protein results in similar biochemical changes suggesting that these proteins function in a common lysosomal pathwayLysosomal dysfunction and impaired autophagy in a novel mouse model deficient for the lysosomal membrane protein Cln7Lysosomal Re-acidification Prevents Lysosphingolipid-Induced Lysosomal Impairment and Cellular ToxicityNiemann-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 brainsSensitivity to lysosome-dependent cell death is directly regulated by lysosomal cholesterol contentAlterations in gene expression in mutant amyloid precursor protein transgenic mice lacking Niemann-Pick type C1 proteinOverexpression of the IGF-II/M6P receptor in mouse fibroblast cell lines differentially alters expression profiles of genes involved in Alzheimer's disease-related pathologyAmino acid substitution in NPC1 that abolishes cholesterol binding reproduces phenotype of complete NPC1 deficiency in mice.Structure-Based Peptide Design to Modulate Amyloid Beta Aggregation and Reduce CytotoxicityCathepsin B overexpression due to acid sphingomyelinase ablation promotes liver fibrosis in Niemann-Pick disease.Heat Shock Protein Beta-1 Modifies Anterior to Posterior Purkinje Cell Vulnerability in a Mouse Model of Niemann-Pick Type C DiseaseCln1 gene disruption in mice reveals a common pathogenic link between two of the most lethal childhood neurodegenerative lysosomal storage disordersCholinergic abnormalities, endosomal alterations and up-regulation of nerve growth factor signaling in Niemann-Pick type C disease.Microarray expression analysis and identification of serum biomarkers for Niemann-Pick disease, type C1.Loss of Cathepsin B and L Leads to Lysosomal Dysfunction, NPC-Like Cholesterol Sequestration and Accumulation of the Key Alzheimer's ProteinsGenetic and pharmacological evidence implicates cathepsins in Niemann-Pick C cerebellar degeneration.Impaired proteolysis underlies autophagic dysfunction in Niemann-Pick type C diseaseLysosomal membrane permeability stimulates protein aggregate formation in neurons of a lysosomal diseasePlasma signature of neurological disease in the monogenetic disorder Niemann-Pick Type C.A novel intermediate mucolipidosis II/IIIαβ caused by GNPTAB mutation in the cytosolic N-terminal domain.Defective cholesterol trafficking in Niemann-Pick C-deficient cells.Cysteine cathepsins in neurological disorders.Insulin-Like Growth Factor-II/Cation-Independent Mannose 6-Phosphate Receptor in Neurodegenerative Diseases.Lysosome and endoplasmic reticulum quality control pathways in Niemann-Pick type C disease.Identification of lysosomal Npc1-binding proteins: Cathepsin D activity is regulated by NPC1.Plasma cathepsin D correlates with histological classifications of fatty liver disease in adults and responds to intervention.Role of cathepsin D in U18666A-induced neuronal cell death: potential implication in Niemann-Pick type C disease pathogenesis.Quantitative Analysis of the Proteome Response to the Histone Deacetylase Inhibitor (HDACi) Vorinostat in Niemann-Pick Type C1 disease.The Effects of Extracellular Serum Concentration on APP Processing in Npc1-Deficient APP-Overexpressing N2a Cells.Effects of cholesterol transport inhibitor U18666A on APP metabolism in rat primary astrocytes.Cerebellar long-term depression is deficient in Niemann-Pick type C disease mice.Spatial and temporal expression of lysosomal acid phosphatase 2 (ACP2) reveals dynamic patterning of the mouse cerebellar cortex.Plasma Cathepsin D Levels: A Novel Tool to Predict Pediatric Hepatic Inflammation
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P2860
Increased activity and altered subcellular distribution of lysosomal enzymes determine neuronal vulnerability in Niemann-Pick type C1-deficient mice.
description
2009 nî lūn-bûn
@nan
2009年の論文
@ja
2009年論文
@yue
2009年論文
@zh-hant
2009年論文
@zh-hk
2009年論文
@zh-mo
2009年論文
@zh-tw
2009年论文
@wuu
2009年论文
@zh
2009年论文
@zh-cn
name
Increased activity and altered ...... n-Pick type C1-deficient mice.
@en
Increased activity and altered ...... n-Pick type C1-deficient mice.
@nl
type
label
Increased activity and altered ...... n-Pick type C1-deficient mice.
@en
Increased activity and altered ...... n-Pick type C1-deficient mice.
@nl
prefLabel
Increased activity and altered ...... n-Pick type C1-deficient mice.
@en
Increased activity and altered ...... n-Pick type C1-deficient mice.
@nl
P2093
P2860
P1476
Increased activity and altered ...... n-Pick type C1-deficient mice.
@en
P2093
Anitha Kodam
Asha Amritraj
Jean E Vance
Kyle Peake
Satyabrata Kar
P2860
P304
P356
10.2353/AJPATH.2009.081096
P407
P577
2009-11-05T00:00:00Z