Toxicity of glucosylsphingosine (glucopsychosine) to cultured neuronal cells: a model system for assessing neuronal damage in Gaucher disease type 2 and 3.
about
Systemic delivery of a glucosylceramide synthase inhibitor reduces CNS substrates and increases lifespan in a mouse model of type 2 Gaucher diseaseLysosomal Re-acidification Prevents Lysosphingolipid-Induced Lysosomal Impairment and Cellular ToxicityMurine models of acute neuronopathic Gaucher disease.Multi-system disorders of glycosphingolipid and ganglioside metabolismSubstrate compositional variation with tissue/region and Gba1 mutations in mouse models--implications for Gaucher diseaseProperties of neurons derived from induced pluripotent stem cells of Gaucher disease type 2 patient fibroblasts: potential role in neuropathology.Progression of Behavioral and CNS Deficits in a Viable Murine Model of Chronic Neuronopathic Gaucher Disease.Elevated globotriaosylsphingosine is a hallmark of Fabry disease.Ambroxol chaperone therapy for neuronopathic Gaucher disease: A pilot study.Biochemical response to substrate reduction therapy versus enzyme replacement therapy in Gaucher disease type 1 patientsElevated plasma glucosylsphingosine in Gaucher disease: relation to phenotype, storage cell markers, and therapeutic responseCNS-accessible Inhibitor of Glucosylceramide Synthase for Substrate Reduction Therapy of Neuronopathic Gaucher Disease.Upregulation of proinflammatory cytokines in the fetal brain of the Gaucher mouse.Autophagy-lysosome pathway associated neuropathology and axonal degeneration in the brains of alpha-galactosidase A-deficient mice.Characterization and application of a disease-cell model for a neurodegenerative lysosomal disease.Neuropathic Gaucher disease.Synaptic failure: The achilles tendon of sphingolipidosesGlucosylsphingosine promotes α-synuclein pathology in mutant GBA-associated Parkinson's disease.Identification of a feedback loop involving β-glucosidase 2 and its product sphingosine sheds light on the molecular mechanisms in Gaucher disease.Parkinson's disease: acid-glucocerebrosidase activity and alpha-synuclein clearance.Development of a model system for neuronal dysfunction in Fabry disease.Neuronopathic Gaucher disease in the mouse: viable combined selective saposin C deficiency and mutant glucocerebrosidase (V394L) mice with glucosylsphingosine and glucosylceramide accumulation and progressive neurological deficitsNeuronal accumulation of glucosylceramide in a mouse model of neuronopathic Gaucher disease leads to neurodegeneration.Glucosylsphingosine is a key biomarker of Gaucher disease.Glucosylsphingosine Causes Hematological and Visceral Changes in Mice-Evidence for a Pathophysiological Role in Gaucher Disease.Reduced cerebral vascularization in experimental neuronopathic Gaucher disease.A convenient approach to facilitate monitoring Gaucher disease progression and therapeutic response.Glycosphingolipid levels and glucocerebrosidase activity are altered in normal aging of the mouse brain.
P2860
Q28482370-E41A5F38-540F-42F1-8B83-517FFC1161DBQ28555118-D4D9107B-94DA-4CCD-905D-8B44118F457EQ30480646-F0F6A3F5-7EA5-4CB6-9295-B37CB6CB3233Q33902037-44FD9A5B-2C8C-41A9-AB9C-4F57CC9AA95FQ34631690-DBF79549-2AD0-47E5-B1EC-1CB13EC64363Q35589803-B523C0C7-555B-40C8-A4D6-ABC63C3C95BDQ36123828-B30818FB-DBC8-4154-A73E-DE4A7B655CC8Q36498349-7CC411D2-D11A-4A70-84DA-74287F3FBD7DQ36639634-0EA74514-D2B8-4A7C-9EE5-EAE8BD240647Q36725221-1386D2D3-1E0E-42FA-942D-586199FDCC87Q36938544-3FC6F8EC-1665-45D4-8DA2-702E77455F79Q37043500-A1D01332-BD7E-413C-8401-9E64822A9543Q37313215-07731243-BD24-47AE-8DF9-999BA04100C9Q37600470-0BFEC171-2A4E-4325-BC21-A6023A57D864Q37624819-34627566-34D9-42B0-B9BE-43879F0E004DQ37827417-AF78443E-569C-407C-A0F5-CD011E186C88Q38559219-DC820AF1-B390-4C13-8410-39EFED9080BFQ38605208-44037BBF-9901-4716-9507-2B9FE4EE50BFQ38713961-BF962F39-CA2D-462C-B603-21CA078DA7DFQ38727638-58E040B2-D9C5-4FE2-8356-2411D6B7778CQ38754923-4E7C3CFA-842C-49D9-9801-89D47D9A1560Q39357071-EC53E6DF-98D8-4A0C-B17E-8E2703FEE931Q41887691-35284347-9B9E-4177-AC33-D8FF1577B9ACQ43008509-8BE38406-B577-445F-A82C-AA8799758476Q47101409-139B365F-7B07-480C-9979-E9DFFE24A3F6Q48032967-40A443B9-C541-48F8-82C0-6A3EBF46EA9BQ48152784-2990D6A0-102F-487C-8A54-A9972BAF633EQ55363665-32E7D94E-250C-4586-BFC5-17A55E74CCF5
P2860
Toxicity of glucosylsphingosine (glucopsychosine) to cultured neuronal cells: a model system for assessing neuronal damage in Gaucher disease type 2 and 3.
description
2003 nî lūn-bûn
@nan
2003年の論文
@ja
2003年論文
@yue
2003年論文
@zh-hant
2003年論文
@zh-hk
2003年論文
@zh-mo
2003年論文
@zh-tw
2003年论文
@wuu
2003年论文
@zh
2003年论文
@zh-cn
name
Toxicity of glucosylsphingosin ...... Gaucher disease type 2 and 3.
@en
type
label
Toxicity of glucosylsphingosin ...... Gaucher disease type 2 and 3.
@en
prefLabel
Toxicity of glucosylsphingosin ...... Gaucher disease type 2 and 3.
@en
P2093
P1476
Toxicity of glucosylsphingosin ...... Gaucher disease type 2 and 3.
@en
P2093
Blusztajn JK
Herkenham M
Kaneski CR
Sandhoff K
Schueler UH
P304
P356
10.1016/J.NBD.2003.08.016
P577
2003-12-01T00:00:00Z