Pharmacological Chaperones and Coenzyme Q10 Treatment Improves Mutant β-Glucocerebrosidase Activity and Mitochondrial Function in Neuronopathic Forms of Gaucher Disease
出版年份 2015 全文链接
标题
Pharmacological Chaperones and Coenzyme Q10 Treatment Improves Mutant β-Glucocerebrosidase Activity and Mitochondrial Function in Neuronopathic Forms of Gaucher Disease
作者
关键词
-
出版物
Scientific Reports
Volume 5, Issue 1, Pages -
出版商
Springer Nature
发表日期
2015-06-05
DOI
10.1038/srep10903
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注意:仅列出部分参考文献,下载原文获取全部文献信息。- Clinical applications of coenzyme Q₁₀
- (2014) Juan Garrido-Maraver Frontiers in Bioscience-Landmark
- Targeted delivery of pharmacological chaperones for Gaucher disease to macrophages by a mannosylated cyclodextrin carrier
- (2014) Julio Rodríguez-Lavado et al. ORGANIC & BIOMOLECULAR CHEMISTRY
- Mitochondria and Quality Control Defects in a Mouse Model of Gaucher Disease—Links to Parkinson’s Disease
- (2013) Laura D. Osellame et al. Cell Metabolism
- Bicyclic Derivatives ofL-Idonojirimycin as Pharmacological Chaperones for Neuronopathic Forms of Gaucher Disease
- (2013) Pilar Alfonso et al. CHEMBIOCHEM
- Neuronal accumulation of glucosylceramide in a mouse model of neuronopathic Gaucher disease leads to neurodegeneration
- (2013) T. Farfel-Becker et al. HUMAN MOLECULAR GENETICS
- Treatment effect of coenzyme Q10 and an antioxidant cocktail in fibroblasts of patients with Sanfilippo disease
- (2013) Leslie Matalonga et al. JOURNAL OF INHERITED METABOLIC DISEASE
- Pharmacological Chaperones as Therapeutics for Lysosomal Storage Diseases
- (2013) Robert E. Boyd et al. JOURNAL OF MEDICINAL CHEMISTRY
- A Multicenter Study of Glucocerebrosidase Mutations in Dementia With Lewy Bodies
- (2013) Michael A. Nalls et al. JAMA Neurology
- Autophagy in lysosomal storage disorders
- (2012) Andrew P. Lieberman et al. Autophagy
- Ambroxol as a pharmacological chaperone for mutant glucocerebrosidase
- (2012) Inna Bendikov-Bar et al. BLOOD CELLS MOLECULES AND DISEASES
- Tuning glycosidase inhibition through aglycone interactions: pharmacological chaperones for Fabry disease and GM1 gangliosidosis
- (2012) M. Aguilar-Moncayo et al. CHEMICAL COMMUNICATIONS
- Impaired parkin-mediated mitochondrial targeting to autophagosomes differentially contributes to tissue pathology in lysosomal storage diseases
- (2012) Raquel de Pablo-Latorre et al. HUMAN MOLECULAR GENETICS
- Lysosomal storage disorders: The cellular impact of lysosomal dysfunction
- (2012) Frances M. Platt et al. JOURNAL OF CELL BIOLOGY
- Potent Aminocyclitol Glucocerebrosidase Inhibitors are Subnanomolar Pharmacological Chaperones for Treating Gaucher Disease
- (2012) Ana Trapero et al. JOURNAL OF MEDICINAL CHEMISTRY
- Ceramide targets autophagosomes to mitochondria and induces lethal mitophagy
- (2012) R David Sentelle et al. Nature Chemical Biology
- Pharmacological chaperone therapy for Gaucher disease: a patent review
- (2011) Juan M Benito et al. EXPERT OPINION ON THERAPEUTIC PATENTS
- Secondary coenzyme Q10 deficiency triggers mitochondria degradation by mitophagy in MELAS fibroblasts
- (2011) David Cotán et al. FASEB JOURNAL
- Bicyclic (galacto)nojirimycin analogues as glycosidase inhibitors: Effect of structural modifications in their pharmacological chaperone potential towards β-glucocerebrosidase
- (2011) Matilde Aguilar-Moncayo et al. ORGANIC & BIOMOLECULAR CHEMISTRY
- Mitochondria and the Autophagy-Inflammation-Cell Death Axis in Organismal Aging
- (2011) D. R. Green et al. SCIENCE
- Exploring the link between glucocerebrosidase mutations and parkinsonism
- (2011) Wendy Westbroek et al. TRENDS IN MOLECULAR MEDICINE
- The proteome of lysosomes
- (2010) Bernd A. Schröder et al. PROTEOMICS
- Regulation of autophagy by ROS: physiology and pathology
- (2010) Ruth Scherz-Shouval et al. TRENDS IN BIOCHEMICAL SCIENCES
- Mutations for Gaucher Disease Confer High Susceptibility to Parkinson Disease
- (2009) Jun Mitsui et al. ARCHIVES OF NEUROLOGY
- Coenzyme Q deficiency triggers mitochondria degradation by mitophagy
- (2009) Ángeles Rodríguez-Hernández et al. Autophagy
- Treating lysosomal storage diseases with pharmacological chaperones: from concept to clinics
- (2009) Giancarlo Parenti EMBO Molecular Medicine
- Multicenter Analysis of Glucocerebrosidase Mutations in Parkinson's Disease
- (2009) E. Sidransky et al. NEW ENGLAND JOURNAL OF MEDICINE
- Movement Disorders Caused by Medical Disease
- (2009) Brandon Barton et al. SEMINARS IN NEUROLOGY
- Randomized, controlled trial of miglustat in Gaucher's disease type 3
- (2008) Raphael Schiffmann et al. ANNALS OF NEUROLOGY
- Molecular Basis for β-Glucosidase Inhibition by Ring-Modified Calystegine Analogues
- (2008) Matilde Aguilar et al. CHEMBIOCHEM
- Glucosylceramide accumulation is not confined to the lysosome in fibroblasts from patients with Gaucher disease
- (2008) Maria Fuller et al. MOLECULAR GENETICS AND METABOLISM
- The in-depth evaluation of suspected mitochondrial disease
- (2008) Richard H. Haas et al. MOLECULAR GENETICS AND METABOLISM
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