Elevation of intracellular glucosylceramide levels results in an increase in endoplasmic reticulum density and in functional calcium stores in cultured neurons.
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Huntingtin and huntingtin-associated protein 1 influence neuronal calcium signaling mediated by inositol-(1,4,5) triphosphate receptor type 1Klotho-related protein is a novel cytosolic neutral beta-glycosylceramidaseChemical and biological approaches for adapting proteostasis to ameliorate protein misfolding and aggregation diseases: progress and prognosisThe delicate balance between secreted protein folding and endoplasmic reticulum-associated degradation in human physiologyLysosome and calcium dysregulation in Alzheimer's disease: partners in crimeInhibition of calcium uptake via the sarco/endoplasmic reticulum Ca2+-ATPase in a mouse model of Sandhoff disease and prevention by treatment with N-butyldeoxynojirimycinAberrant Ca2+ handling in lysosomal storage disordersCommon and uncommon pathogenic cascades in lysosomal storage diseasesGaucher disease ascertained through a Parkinson's center: imaging and clinical characterization.Age-dependent therapeutic effect of memantine in a mouse model of juvenile Batten diseaseType 2 Gaucher disease: phenotypic variation and genotypic heterogeneity.Gaucher disease: pathological mechanisms and modern management.Regulating survival and development in the retina: key roles for simple sphingolipids.Remodeling the proteostasis network to rescue glucocerebrosidase variants by inhibiting ER-associated degradation and enhancing ER folding.Endoplasmic reticulum Ca(2+) handling in excitable cells in health and diseaseUnbiased Cell-based Screening in a Neuronal Cell Model of Batten Disease Highlights an Interaction between Ca2+ Homeostasis, Autophagy, and CLN3 Protein Function.Role of endolysosomes in HIV-1 Tat-induced neurotoxicityGaucher disease gene GBA functions in immune regulation.Progression of Behavioral and CNS Deficits in a Viable Murine Model of Chronic Neuronopathic Gaucher Disease.Storage solutions: treating lysosomal disorders of the brain.Neuroinflammatory paradigms in lysosomal storage diseases.Neuronopathic Gaucher disease: dysregulated mRNAs and miRNAs in brain pathogenesis and effects of pharmacologic chaperone treatment in a mouse modelTargeted Plasma Membrane Delivery of a Hydrophobic Cargo Encapsulated in a Liquid Crystal Nanoparticle Carrier.The cognitive profile of type 1 Gaucher disease patients.Acidic Ca2+ stores in neurodegeneration.A new glucocerebrosidase-deficient neuronal cell model provides a tool to probe pathophysiology and therapeutics for Gaucher disease.β-Glucosidase 2 (GBA2) activity and imino sugar pharmacology.Covalent inhibitors of glycosidases and their applications in biochemistry and biology.Upregulation of proinflammatory cytokines in the fetal brain of the Gaucher mouse.Accumulation of ordered ceramide-cholesterol domains in farber disease fibroblasts.Strategies for delivery of therapeutics into the central nervous system for treatment of lysosomal storage disorders.Endoplasmic-reticulum calcium depletion and disease.Lysosomal storage disorders and Parkinson's disease: Gaucher disease and beyond.The cellular pathology of lysosomal diseases.Gaucher disease paradigm: from ERAD to comorbidity.Lysosomal storage diseases--the horizon expands.Endoplasmic reticulum and lysosomal Ca²⁺ stores are remodelled in GBA1-linked Parkinson disease patient fibroblasts.Chronic pain in Gaucher disease: skeletal or neuropathic origin?Current and Novel Aspects on the Non-lysosomal β-Glucosylceramidase GBA2.Pharmaceutical Chaperones and Proteostasis Regulators in the Therapy of Lysosomal Storage Disorders: Current Perspective and Future Promises.
P2860
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P2860
Elevation of intracellular glucosylceramide levels results in an increase in endoplasmic reticulum density and in functional calcium stores in cultured neurons.
description
1999 nî lūn-bûn
@nan
1999年の論文
@ja
1999年学术文章
@wuu
1999年学术文章
@zh-cn
1999年学术文章
@zh-hans
1999年学术文章
@zh-my
1999年学术文章
@zh-sg
1999年學術文章
@yue
1999年學術文章
@zh
1999年學術文章
@zh-hant
name
Elevation of intracellular glu ...... um stores in cultured neurons.
@en
Elevation of intracellular glu ...... um stores in cultured neurons.
@nl
type
label
Elevation of intracellular glu ...... um stores in cultured neurons.
@en
Elevation of intracellular glu ...... um stores in cultured neurons.
@nl
prefLabel
Elevation of intracellular glu ...... um stores in cultured neurons.
@en
Elevation of intracellular glu ...... um stores in cultured neurons.
@nl
P2093
P2860
P356
P1476
Elevation of intracellular glu ...... um stores in cultured neurons.
@en
P2093
P2860
P304
21673-21678
P356
10.1074/JBC.274.31.21673
P407
P577
1999-07-01T00:00:00Z