Metabolism of Glycosphingolipids and Their Role in the Pathophysiology of Lysosomal Storage Disorders
出版年份 2020 全文链接
标题
Metabolism of Glycosphingolipids and Their Role in the Pathophysiology of Lysosomal Storage Disorders
作者
关键词
-
出版物
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
Volume 21, Issue 18, Pages 6881
出版商
MDPI AG
发表日期
2020-09-19
DOI
10.3390/ijms21186881
参考文献
相关参考文献
注意：仅列出部分参考文献，下载原文获取全部文献信息。- Glucocerebrosidases catalyze a transgalactosylation reaction that yields a newly identified brain sterol metabolite, galactosylated cholesterol
- (2020) Hisako Akiyama et al. JOURNAL OF BIOLOGICAL CHEMISTRY
- The process of Lewy body formation, rather than simply α-synuclein fibrillization, is one of the major drivers of neurodegeneration
- (2020) Anne-Laure Mahul-Mellier et al. PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
- C6-ceramide induces salivary adenoid cystic carcinoma cell apoptosis via IP3R-activated UPR and UPR-independent pathways
- (2020) Ling Qiu et al. BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS
- Mechanism of Secondary Ganglioside and Lipid Accumulation in Lysosomal Disease
- (2020) Bernadette Breiden et al. INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
- LRP1 is a master regulator of tau uptake and spread
- (2020) Jennifer N. Rauch et al. NATURE
- Emerging roles for human glycolipid transfer protein superfamily members in the regulation of autophagy, inflammation, and cell death
- (2020) Shrawan K. Mishra et al. PROGRESS IN LIPID RESEARCH
- Brain delivery and activity of a lysosomal enzyme using a blood-brain barrier transport vehicle in mice
- (2020) Julie C. Ullman et al. Science Translational Medicine
- Verotoxin Receptor-Based Pathology and Therapies
- (2020) Clifford Lingwood Frontiers in Cellular and Infection Microbiology
- Novel Drug Candidates Improve Ganglioside Accumulation and Neural Dysfunction in GM1 Gangliosidosis Models with Autophagy Activation
- (2020) Ryutaro Kajihara et al. Stem Cell Reports
- Altered Sphingolipids Metabolism Damaged Mitochondrial Functions: Lessons Learned From Gaucher and Fabry Diseases
- (2020) Margarita Ivanova Journal of Clinical Medicine
- Mice deficient in GM1 manifest both motor and non-motor symptoms of Parkinson's disease; successful treatment with synthetic GM1 ganglioside
- (2020) Gusheng Wu et al. EXPERIMENTAL NEUROLOGY
- Lysosomal Storage Disorders Shed Light on Lysosomal Dysfunction in Parkinson’s Disease
- (2020) Shani Blumenreich et al. INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
- Lysosome-targeted β-galactosidase negatively regulates neuraminidase 1 (NEU1) and promotes NEU1 deficiency in GM1 gangliosidosis.
- (2020) Amanda R Luu et al. JOURNAL OF BIOLOGICAL CHEMISTRY
- Detection of GM1‐Gangliosidosis in Newborn Dried Blood Spots by Enzyme and Biomarker Assays Using Tandem Mass Spectrometry
- (2020) Peiling Su et al. JOURNAL OF INHERITED METABOLIC DISEASE
- Mitophagy, Mitochondrial Homeostasis, and Cell Fate
- (2020) Kaili Ma et al. Frontiers in Cell and Developmental Biology
- Hematopoietic stem cell transplantation in mucopolysaccharidosis type IIIA: A case description and comparison with a genotype-matched control group
- (2020) Anja F. Köhn et al. Molecular Genetics and Metabolism Reports
- Impaired autophagic and mitochondrial functions are partially restored by ERT in Gaucher and Fabry diseases
- (2019) Margarita M. Ivanova et al. PLoS One
- Extracellular aggregated alpha synuclein primarily triggers lysosomal dysfunction in neural cells prevented by trehalose
- (2019) Anna-Carin Hoffmann et al. Scientific Reports
- FACTs Fabry gene therapy clinical trial: Two-year data
- (2019) Jeffrey A. Medin et al. MOLECULAR GENETICS AND METABOLISM
- New era of research on cancer‐associated glycosphingolipids
- (2019) Koichi Furukawa et al. CANCER SCIENCE
- Ganglioside GM1 promotes contact inhibition of growth by regulating the localization of epidermal growth factor receptor from glycosphingolipid‐enriched microdomain to caveolae
- (2019) Dinghao Zhuo et al. CELL PROLIFERATION
- Membrane lipids and their degradation compounds control GM2 catabolism at intralysosomal luminal vesicles
- (2019) Susi Anheuser et al. JOURNAL OF LIPID RESEARCH
- The UDP-Glycosyltransferase (UGT) Superfamily: New Members, New Functions, and Novel Paradigms
- (2019) Robyn Meech et al. PHYSIOLOGICAL REVIEWS
- Substrate Reduction Therapy for Sandhoff Disease through Inhibition of Glucosylceramide Synthase Activity
- (2019) John Marshall et al. MOLECULAR THERAPY
- Anti-Siglec-1 antibodies block Ebola viral uptake and decrease cytoplasmic viral entry
- (2019) Daniel Perez-Zsolt et al. Nature Microbiology
- Gangliosides: Treatment Avenues in Neurodegenerative Disease
- (2019) Pierre J. Magistretti et al. Frontiers in Neurology
- Human genome-edited hematopoietic stem cells phenotypically correct Mucopolysaccharidosis type I
- (2019) Natalia Gomez-Ospina et al. Nature Communications
- GM1 Ganglioside role in the interaction of Alpha-synuclein with lipid membranes: Morphology and structure
- (2019) F. Perissinotto et al. BIOPHYSICAL CHEMISTRY
- Acid Sphingomyelinase Deficiency Ameliorates Farber Disease
- (2019) Nadine Beckmann et al. INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
- LRRK2 kinase activity regulates lysosomal glucocerebrosidase in neurons derived from Parkinson’s disease patients
- (2019) Daniel Ysselstein et al. Nature Communications
- Parkinson’s disease recovery by GM1 oligosaccharide treatment in the B4galnt1+/− mouse model
- (2019) Elena Chiricozzi et al. Scientific Reports
- Long-Term Functional Outcomes after Hematopoietic Stem Cell Transplant for Early Infantile Krabbe Disease
- (2018) Heather Allewelt et al. BIOLOGY OF BLOOD AND MARROW TRANSPLANTATION
- Genetics and Therapies for GM2 Gangliosidosis
- (2018) Maria Begona Cachon-Gonzalez et al. CURRENT GENE THERAPY
- Emerging concepts of ganglioside metabolism
- (2018) Roger Sandhoff et al. FEBS LETTERS
- Amyloid β fibrils assembled on ganglioside-enriched membranes contain both parallel β-sheets and turns
- (2018) Teruhiko Matsubara et al. JOURNAL OF BIOLOGICAL CHEMISTRY
- Diseases of ganglioside biosynthesis: An expanding group of congenital disorders of glycosylation
- (2018) Marco Trinchera et al. MOLECULAR GENETICS AND METABOLISM
- Acid ceramidase deficiency: Farber disease and SMA-PME
- (2018) Fabian P. S. Yu et al. Orphanet Journal of Rare Diseases
- Altered expression of genes involved in ganglioside biosynthesis in substantia nigra neurons in Parkinson’s disease
- (2018) Jay S. Schneider PLoS One
- A common mechanism of proteasome impairment by neurodegenerative disease-associated oligomers
- (2018) Tiffany A. Thibaudeau et al. Nature Communications
- Structural basis for the activation of acid ceramidase
- (2018) Ahmad Gebai et al. Nature Communications
- A patient-specific induced pluripotent stem cell model for West syndrome caused by ST3GAL3 deficiency
- (2018) Laura van Diepen et al. EUROPEAN JOURNAL OF HUMAN GENETICS
- My journey in the discovery of nucleotide sugar transporters of the Golgi apparatus
- (2018) Carlos B. Hirschberg JOURNAL OF BIOLOGICAL CHEMISTRY
- Complex formation of sphingomyelin synthase 1 with glucosylceramide synthase increases sphingomyelin and decreases glucosylceramide levels
- (2018) Yasuhiro Hayashi et al. JOURNAL OF BIOLOGICAL CHEMISTRY
- Lactosylceramide synthases encoded by B4galt5 and 6 genes are pivotal for neuronal generation and myelin formation in mice
- (2018) Toru Yoshihara et al. PLoS Genetics
- Glycosphingolipid metabolism in cell fate specification
- (2018) Domenico Russo et al. JOURNAL OF CELL SCIENCE
- A validated HPLC-MS/MS method for estimating the concentration of the ganglioside, GD2, in human plasma or serum
- (2018) C.M. Busch et al. JOURNAL OF CHROMATOGRAPHY B-ANALYTICAL TECHNOLOGIES IN THE BIOMEDICAL AND LIFE SCIENCES
- Loss of Enzyme Activity in Mutated B4GALNT1 Gene Products in Patients with Hereditary Spastic Paraplegia Results in Relatively Mild Neurological Disorders: Similarity with Phenotypes of B4galnt1 Knockout Mice
- (2018) Robiul H. Bhuiyan et al. NEUROSCIENCE
- Efficacy of a Bicistronic Vector for Correction of Sandhoff Disease in a Mouse Model
- (2018) Evan Woodley et al. Molecular Therapy-Methods & Clinical Development
- Upregulation of human glycolipid transfer protein (GLTP) induces necroptosis in colon carcinoma cells
- (2018) Shrawan Kumar Mishra et al. BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR AND CELL BIOLOGY OF LIPIDS
- Gaucher disease: Progress and ongoing challenges
- (2017) Pramod K. Mistry et al. MOLECULAR GENETICS AND METABOLISM
- Proteasome impairment by α-synuclein
- (2017) Lisa Zondler et al. PLoS One
- Influenza A virus hemagglutinin and neuraminidase act as novel motile machinery
- (2017) Tatsuya Sakai et al. Scientific Reports
- A new glucocerebrosidase-deficient neuronal cell model provides a tool to probe pathophysiology and therapeutics for Gaucher disease
- (2016) Wendy Westbroek et al. Disease Models & Mechanisms
- Systemic Gene Transfer of a Hexosaminidase Variant Using an scAAV9.47 Vector Corrects GM2Gangliosidosis in Sandhoff Mice
- (2016) Karlaina J.L. Osmon et al. HUMAN GENE THERAPY
- The role of sphingolipids in neuronal plasticity of the brain
- (2016) Sandro Sonnino et al. JOURNAL OF NEUROCHEMISTRY
- Activation of -Glucocerebrosidase Reduces Pathological -Synuclein and Restores Lysosomal Function in Parkinson's Patient Midbrain Neurons
- (2016) J. R. Mazzulli et al. JOURNAL OF NEUROSCIENCE
- Enzyme replacement for GM1-gangliosidosis: Uptake, lysosomal activation, and cellular disease correction using a novel β-galactosidase:RTB lectin fusion
- (2016) Jose Condori et al. MOLECULAR GENETICS AND METABOLISM
- Chemical properties of lipids strongly affect the kinetics of the membrane-induced aggregation of α-synuclein
- (2016) Céline Galvagnion et al. PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
- Construction of a hybrid β-hexosaminidase subunit capable of forming stable homodimers that hydrolyze GM2 ganglioside in vivo
- (2016) Michael B Tropak et al. Molecular Therapy-Methods & Clinical Development
- Molecular species analysis of monosialogangliosides from sea urchin Strongylocentrotus nudus by RPLC-ESI-MS/MS
- (2015) Pei-Xu Cong et al. FOOD CHEMISTRY
- Systemic AAV9 gene transfer in adult GM1 gangliosidosis mice reduces lysosomal storage in CNS and extends lifespan
- (2015) Cara M. Weismann et al. HUMAN MOLECULAR GENETICS
- Long-Term Correction of Sandhoff Disease Following Intravenous Delivery of rAAV9 to Mouse Neonates
- (2015) Jagdeep S Walia et al. MOLECULAR THERAPY
- Lipid vesicles trigger α-synuclein aggregation by stimulating primary nucleation
- (2015) Céline Galvagnion et al. Nature Chemical Biology
- Ganglioside and Non-ganglioside Mediated Host Responses to the Mouse Polyomavirus
- (2015) John You et al. PLoS Pathogens
- A new liquid chromatography/tandem mass spectrometry method for quantification of gangliosides in human plasma
- (2014) Qianyang Huang et al. ANALYTICAL BIOCHEMISTRY
- High sphingomyelin levels induce lysosomal damage and autophagy dysfunction in Niemann Pick disease type A
- (2014) E Gabandé-Rodríguez et al. CELL DEATH AND DIFFERENTIATION
- Congenital disorder of fucosylation type 2c (LADII) presenting with short stature and developmental delay with minimal adhesion defect
- (2014) Andrew Dauber et al. HUMAN MOLECULAR GENETICS
- GM1 ganglioside enhances Ret signaling in striatum
- (2014) Erin N. Newburn et al. JOURNAL OF NEUROCHEMISTRY
- Neuronal inclusions of α-synuclein contribute to the pathogenesis of Krabbe disease
- (2014) Benjamin R Smith et al. JOURNAL OF PATHOLOGY
- Virus Particle Release from Glycosphingolipid-Enriched Microdomains Is Essential for Dendritic Cell-Mediated Capture and Transfer of HIV-1 and Henipavirus
- (2014) H. Akiyama et al. JOURNAL OF VIROLOGY
- GBA2-Encoded β-Glucosidase Activity Is Involved in the Inflammatory Response to Pseudomonas aeruginosa
- (2014) Nicoletta Loberto et al. PLoS One
- Protein aggregation can inhibit clathrin-mediated endocytosis by chaperone competition
- (2014) A. Yu et al. PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
- Sphingolipid Metabolism and Interorganellar Transport: Localization of Sphingolipid Enzymes and Lipid Transfer Proteins
- (2014) Toshiyuki Yamaji et al. TRAFFIC
- HIV-1 Capture and Transmission by Dendritic Cells: The Role of Viral Glycolipids and the Cellular Receptor Siglec-1
- (2014) Nuria Izquierdo-Useros et al. PLoS Pathogens
- The ubiquitin-proteasome system in neurodegenerative diseases: precipitating factor, yet part of the solution
- (2014) Nico P. Dantuma et al. Frontiers in Molecular Neuroscience
- Crossroads between Bacterial and Mammalian Glycosyltransferases
- (2014) Inka Brockhausen Frontiers in Immunology
- Loss of Function of Glucocerebrosidase GBA2 Is Responsible for Motor Neuron Defects in Hereditary Spastic Paraplegia
- (2013) Elodie Martin et al. AMERICAN JOURNAL OF HUMAN GENETICS
- Alteration of Ganglioside Biosynthesis Responsible for Complex Hereditary Spastic Paraplegia
- (2013) Amir Boukhris et al. AMERICAN JOURNAL OF HUMAN GENETICS
- Mutations in B4GALNT1 (GM2 synthase) underlie a new disorder of ganglioside biosynthesis
- (2013) Gaurav V. Harlalka et al. BRAIN
- Lysosomal multienzyme complex: pros and cons of working together
- (2013) Erik J. Bonten et al. CELLULAR AND MOLECULAR LIFE SCIENCES
- Lysosomal Membrane Permeability Stimulates Protein Aggregate Formation in Neurons of a Lysosomal Disease
- (2013) M. C. Micsenyi et al. JOURNAL OF NEUROSCIENCE
- Gangliosides and Gangliosidoses: Principles of Molecular and Metabolic Pathogenesis
- (2013) K. Sandhoff et al. JOURNAL OF NEUROSCIENCE
- Chaperone Therapy for GM2 Gangliosidosis: Effects of Pyrimethamine on β-Hexosaminidase Activity in Sandhoff Fibroblasts
- (2013) Elena Chiricozzi et al. MOLECULAR NEUROBIOLOGY
- Differential Distribution of Major Brain Gangliosides in the Adult Mouse Central Nervous System
- (2013) Katarina Vajn et al. PLoS One
- Biosynthesis and biological function of sulfoglycolipids
- (2013) Koichi HONKE PROCEEDINGS OF THE JAPAN ACADEMY SERIES B-PHYSICAL AND BIOLOGICAL SCIENCES
- Lentiviral Hematopoietic Stem Cell Gene Therapy Benefits Metachromatic Leukodystrophy
- (2013) A. Biffi et al. SCIENCE
- Lysosomal NEU1 deficiency affects amyloid precursor protein levels and amyloid-β secretion via deregulated lysosomal exocytosis
- (2013) Ida Annunziata et al. Nature Communications
- Cholesterol accelerates the binding of Alzheimer's β-amyloid peptide to ganglioside GM1 through a universal hydrogen-bond-dependent sterol tuning of glycolipid conformation
- (2013) Jacques Fantini et al. Frontiers in Physiology
- Beyond glycoproteins as galectin counterreceptors: tumor-effector T cell growth control via ganglioside GM1
- (2012) Robert W. Ledeen et al. Annals of the New York Academy of Sciences
- Autophagy in lysosomal storage disorders
- (2012) Andrew P. Lieberman et al. Autophagy
- West syndrome caused byST3Gal-IIIdeficiency
- (2012) Simon Edvardson et al. EPILEPSIA
- Role of α-synuclein penetration into the membrane in the mechanisms of oligomer pore formation
- (2012) Igor F. Tsigelny et al. FEBS Journal
- Glycosphingolipids Are Essential for Intestinal Endocytic Function
- (2012) Richard Jennemann et al. JOURNAL OF BIOLOGICAL CHEMISTRY
- Krabbe disease in adults: phenotypic and genotypic update from a series of 11 cases and a review
- (2012) Rabab Debs et al. JOURNAL OF INHERITED METABOLIC DISEASE
- Cell surface associated glycohydrolases in normal and Gaucher disease fibroblasts
- (2012) Massimo Aureli et al. JOURNAL OF INHERITED METABOLIC DISEASE
- A randomized, controlled, delayed start trial of GM1 ganglioside in treated Parkinson's disease patients
- (2012) Jay S. Schneider et al. JOURNAL OF THE NEUROLOGICAL SCIENCES
- HIV-1 incorporation of host-cell-derived glycosphingolipid GM3 allows for capture by mature dendritic cells
- (2012) W. B. Puryear et al. PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
- ST3GAL3 Mutations Impair the Development of Higher Cognitive Functions
- (2011) Hao Hu et al. AMERICAN JOURNAL OF HUMAN GENETICS
- GM1 gangliosidosis and Morquio B disease: An update on genetic alterations and clinical findings
- (2011) Anna Caciotti et al. BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE
- Structural and Morphological Characterization of Aggregated Species of α-Synuclein Induced by Docosahexaenoic Acid
- (2011) Giorgia De Franceschi et al. JOURNAL OF BIOLOGICAL CHEMISTRY
- Crystal Structure of Human β-Galactosidase
- (2011) Umeharu Ohto et al. JOURNAL OF BIOLOGICAL CHEMISTRY
- Crystal Structure of β-Hexosaminidase B in Complex with Pyrimethamine, a Potential Pharmacological Chaperone
- (2011) Katherine S. Bateman et al. JOURNAL OF MEDICINAL CHEMISTRY
- Structures, Biosynthesis, and Functions of Gangliosides-an Overview
- (2011) Robert K. Yu et al. Journal of Oleo Science
- Mice Lacking Major Brain Gangliosides Develop Parkinsonism
- (2011) Gusheng Wu et al. NEUROCHEMICAL RESEARCH
- β4-Galactosyltransferase-5 is a lactosylceramide synthase essential for mouse extra-embryonic development
- (2010) Toshikazu Nishie et al. GLYCOBIOLOGY
- Evidence of lysosomal membrane permeabilization in mucopolysaccharidosis type I: Rupture of calcium and proton homeostasis
- (2010) Vanessa Gonçalves Pereira et al. JOURNAL OF CELLULAR PHYSIOLOGY
- A Fascination with Sugars
- (2010) Stuart Kornfeld MOLECULAR BIOLOGY OF THE CELL
- Hsp70 stabilizes lysosomes and reverts Niemann–Pick disease-associated lysosomal pathology
- (2010) Thomas Kirkegaard et al. NATURE
- Lipid Rafts As a Membrane-Organizing Principle
- (2010) D. Lingwood et al. SCIENCE
- Identification of Hematopoietic Stem Cell-Specific miRNAs Enables Gene Therapy of Globoid Cell Leukodystrophy
- (2010) B. Gentner et al. Science Translational Medicine
- The role of nucleotide sugar transporters in development of eukaryotes
- (2010) Li Liu et al. SEMINARS IN CELL & DEVELOPMENTAL BIOLOGY
- Membrane microdomains and insulin resistance
- (2009) Jin-ichi Inokuchi FEBS LETTERS
- The 1.9 Å Structure of Human α-N-Acetylgalactosaminidase: The Molecular Basis of Schindler and Kanzaki Diseases
- (2009) Nathaniel E. Clark et al. JOURNAL OF MOLECULAR BIOLOGY
- On the mechanism of internalization of α-synuclein into microglia: roles of ganglioside GM1 and lipid raft
- (2009) Ji-Young Park et al. JOURNAL OF NEUROCHEMISTRY
- GM1-Ganglioside Accumulation at the Mitochondria-Associated ER Membranes Links ER Stress to Ca2+-Dependent Mitochondrial Apoptosis
- (2009) Renata Sano et al. MOLECULAR CELL
- Apoptotic Sphingolipid Signaling by Ceramides in Lung Endothelial Cells
- (2008) Terry R. Medler et al. AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY
- Activation of β1,3-N-Acetylglucosaminyltransferase-2 (β3Gn-T2) by β3Gn-T8
- (2008) Akira Seko et al. JOURNAL OF BIOLOGICAL CHEMISTRY
- Anti‐Ganglioside Antibody Induction by Swine (A/NJ/1976/H1N1) and Other Influenza Vaccines: Insights into Vaccine‐Associated Guillain‐Barré Syndrome
- (2008) Irving Nachamkin et al. JOURNAL OF INFECTIOUS DISEASES
- Thematic Review Series: Sphingolipids.Role of ganglioside metabolism in the pathogenesis of Alzheimer's disease—a review,
- (2008) Toshio Ariga et al. JOURNAL OF LIPID RESEARCH
Create your own webinar
Interested in hosting your own webinar? Check the schedule and propose your idea to the Peeref Content Team.
Create NowAsk a Question. Answer a Question.
Quickly pose questions to the entire community. Debate answers and get clarity on the most important issues facing researchers.
Get Started