about
The novel estrogen-induced gene EIG121 regulates autophagy and promotes cell survival under stressArylsulfatase G, a novel lysosomal sulfataseTau deletion exacerbates the phenotype of Niemann-Pick type C mice and implicates autophagy in pathogenesis.Autophagy in lysosomal storage disordersThe cell biology of disease: lysosomal storage disorders: the cellular impact of lysosomal dysfunctionClarifying lysosomal storage diseasesThe pathogenesis of Niemann-Pick type C disease: a role for autophagy?Sanfilippo syndrome: causes, consequences, and treatmentsDevelopment of autophagy inducers in clinical medicineImpairment of homeostasis in lysosomal storage disordersAutophagy and apoptosis in liver injurySkeletal muscle autophagy: a new metabolic regulatorAutophagy and disease: always two sides to a problemAutophagy modulation as a potential therapeutic target for diverse diseasesAutophagy in polyglutamine disease: Imposing order on disorder or contributing to the chaos?Immune-Mediated Inflammation May Contribute to the Pathogenesis of Cardiovascular Disease in Mucopolysaccharidosis Type ISpreading of a prion domain from cell-to-cell by vesicular transport in Caenorhabditis elegansDefective Autophagy, Mitochondrial Clearance and Lipophagy in Niemann-Pick Type B LymphocytesATP13A2 regulates mitochondrial bioenergetics through macroautophagyReduced cathepsins B and D cause impaired autophagic degradation that can be almost completely restored by overexpression of these two proteases in Sap C-deficient fibroblastsDeveloping therapeutic approaches for metachromatic leukodystrophySelective autophagy mediated by autophagic adapter proteinsAutophagy and lysosomal dysfunction as emerging mechanisms of nanomaterial toxicityMacroautophagy and cell responses related to mitochondrial dysfunction, lipid metabolism and unconventional secretion of proteinsEarly neurodegeneration progresses independently of microglial activation by heparan sulfate in the brain of mucopolysaccharidosis IIIB miceMacroautophagy is defective in mucolipin-1-deficient mouse neuronsLysosomal dysfunction and impaired autophagy in a novel mouse model deficient for the lysosomal membrane protein Cln7Niemann-Pick C disease gene mutations and age-related neurodegenerative disordersThe Murine Model of Mucopolysaccharidosis IIIB Develops Cardiopathies over Time Leading to Heart FailureReduced basal autophagy and impaired mitochondrial dynamics due to loss of Parkinson's disease-associated protein DJ-1Mitochondria and quality control defects in a mouse model of Gaucher disease--links to Parkinson's diseaseTFEB links autophagy to lysosomal biogenesisAutophagy gone awry in neurodegenerative diseasesLacritin and other autophagy associated proteins in ocular surface health.Efficacy of a combined intracerebral and systemic gene delivery approach for the treatment of a severe lysosomal storage disorder.High sphingomyelin levels induce lysosomal damage and autophagy dysfunction in Niemann Pick disease type A.Lysosomal adaptation: how the lysosome responds to external cuesMitochondrial autophagy promotes cellular injury in nephropathic cystinosisRecommendations on clinical trial design for treatment of Mucopolysaccharidosis Type III.Autophagic-lysosomal dysregulation downstream of cathepsin B inactivation in human skin fibroblasts exposed to UVA.
P2860
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P2860
description
2008 nî lūn-bûn
@nan
2008 թուականի Յունուարին հրատարակուած գիտական յօդուած
@hyw
2008 թվականի հունվարին հրատարակված գիտական հոդված
@hy
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
name
A block of autophagy in lysosomal storage disorders
@ast
A block of autophagy in lysosomal storage disorders
@en
A block of autophagy in lysosomal storage disorders
@nl
type
label
A block of autophagy in lysosomal storage disorders
@ast
A block of autophagy in lysosomal storage disorders
@en
A block of autophagy in lysosomal storage disorders
@nl
prefLabel
A block of autophagy in lysosomal storage disorders
@ast
A block of autophagy in lysosomal storage disorders
@en
A block of autophagy in lysosomal storage disorders
@nl
P2093
P2860
P50
P3181
P356
P1476
A block of autophagy in lysosomal storage disorders
@en
P2093
Alessandro Fraldi
Carmine Spampanato
Consuelo Venturi
David C Rubinsztein
Luca Jahreiss
Raquel de Pablo
P2860
P304
P3181
P356
10.1093/HMG/DDM289
P407
P577
2007-10-03T00:00:00Z