Dependence of reversibility and progression of mouse neuronopathic Gaucher disease on acid beta-glucosidase residual activity levels.
about
Immunological cells and functions in Gaucher diseaseIsofagomine in vivo effects in a neuronopathic Gaucher disease mouse.Multi-system disorders of glycosphingolipid and ganglioside metabolismUbiquitous transgene expression of the glucosylceramide-synthesizing enzyme accelerates glucosylceramide accumulation and storage cells in a Gaucher disease mouse model.Sustained Systemic Glucocerebrosidase Inhibition Induces Brain α-Synuclein Aggregation, Microglia and Complement C1q Activation in Mice.Progression of Behavioral and CNS Deficits in a Viable Murine Model of Chronic Neuronopathic Gaucher Disease.Neuronopathic Gaucher disease: dysregulated mRNAs and miRNAs in brain pathogenesis and effects of pharmacologic chaperone treatment in a mouse modelCNS-accessible Inhibitor of Glucosylceramide Synthase for Substrate Reduction Therapy of Neuronopathic Gaucher Disease.Animal models for Gaucher disease research.Animal models for metabolic, neuromuscular and ophthalmological rare diseases.Emerging therapeutic targets for Gaucher disease.Induced Pluripotent Stem Cell Modeling of Gaucher's Disease: What Have We Learned?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 deficitsModulating ryanodine receptors with dantrolene attenuates neuronopathic phenotype in Gaucher disease mice.Delineating pathological pathways in a chemically induced mouse model of Gaucher disease.Accumulation and distribution of α-synuclein and ubiquitin in the CNS of Gaucher disease mouse modelsComplement drives glucosylceramide accumulation and tissue inflammation in Gaucher disease.The contribution of mutant GBA to the development of Parkinson disease in Drosophila.
P2860
Q26827675-7CAEDA2C-CD4C-4538-B574-A487C6D621F6Q33886270-9A681203-4C7E-4CB5-9958-6BD2F96C5AA8Q33902037-04C2BB9A-7A37-4DEF-9077-FDEF6171D027Q35536435-66F8A140-DC0F-45C7-B576-77EA6DCDDEC3Q35982198-F59BEAF8-A5B4-4F05-8BF3-08992AF453D9Q36123828-AB7D4850-80D7-4859-9A9E-55905F789D51Q36302109-84956DB3-B77D-47E5-8ECF-99310707C5DBQ37043500-7576AFB2-461D-4D05-B6F9-556B356047A4Q37939781-C4D03CA4-4B05-4F71-8674-BE3990500B8AQ38089518-1DEDB2F1-45E4-49A9-B6D8-C2294DA3673DQ38270518-6DEECA70-E05B-49CA-A0AE-35843B2BD6E8Q39254537-7B97132A-DDAD-4642-9BC8-1482970C6DB9Q39357071-E3847E63-B804-4F05-ABB0-1629E6665FC8Q39364713-18D69158-2244-4AB6-BC58-8FAF3DD64477Q39730152-5B038C0A-7104-4F60-8EEA-7A55EC383C05Q41871999-888E4F2F-F986-4E26-9F0D-1B2D6DA4D80CQ51127427-D6421260-2FC6-426A-801B-23C321D9C227Q52848620-7204746C-D042-4738-827C-91AB3C7B990B
P2860
Dependence of reversibility and progression of mouse neuronopathic Gaucher disease on acid beta-glucosidase residual activity levels.
description
2008 nî lūn-bûn
@nan
2008年の論文
@ja
2008年論文
@yue
2008年論文
@zh-hant
2008年論文
@zh-hk
2008年論文
@zh-mo
2008年論文
@zh-tw
2008年论文
@wuu
2008年论文
@zh
2008年论文
@zh-cn
name
Dependence of reversibility an ...... dase residual activity levels.
@en
type
label
Dependence of reversibility an ...... dase residual activity levels.
@en
prefLabel
Dependence of reversibility an ...... dase residual activity levels.
@en
P2093
P2860
P1476
Dependence of reversibility an ...... dase residual activity levels.
@en
P2093
Brian Quinn
David Witte
Gregory A Grabowski
Joerg Huelsken
Rachel Reboulet
You-Hai Xu
P2860
P304
P356
10.1016/J.YMGME.2008.01.013
P577
2008-03-17T00:00:00Z