Emerging concepts of ganglioside metabolism

Inactivation of ceramide synthase 2 catalytic activity in mice affects transcription of genes involved in lipid metabolism and cell division

(2018) Andreas Bickert et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR AND CELL BIOLOGY OF LIPIDS

Murine Sialidase Neu3 facilitates GM2 degradation and bypass in mouse model of Tay-Sachs disease

(2018) Volkan Seyrantepe et al.   EXPERIMENTAL NEUROLOGY

A Comparative Study of Human Saposins

(2018) María Garrido-Arandia et al.   MOLECULES

Ceramide Synthase Schlank Is a Transcriptional Regulator Adapting Gene Expression to Energy Requirements

(2018) Mariangela Sociale et al.   Cell Reports

Neuraminidases 3 and 4 regulate neuronal function by catabolizing brain gangliosides

(2017) Xuefang Pan et al.   FASEB JOURNAL

Biallelic Mutations in KDSR Disrupt Ceramide Synthesis and Result in a Spectrum of Keratinization Disorders Associated with Thrombocytopenia

(2017) Takuya Takeichi et al.   JOURNAL OF INVESTIGATIVE DERMATOLOGY

Lipids regulate the hydrolysis of membrane bound glucosylceramide by lysosomal β-glucocerebrosidase

(2017) Misbaudeen Abdul-Hammed et al.   JOURNAL OF LIPID RESEARCH

Emptying the stores: lysosomal diseases and therapeutic strategies

(2017) Frances M. Platt   NATURE REVIEWS DRUG DISCOVERY

The role of S-acylation in protein trafficking

(2017) Jose L. Daniotti et al.   TRAFFIC

Concerted regulation of npc2 binding to endosomal/lysosomal membranes by bis(monoacylglycero)phosphate and sphingomyelin

(2017) Giray Enkavi et al.   PLoS Computational Biology

Human Sialidase Neu3 is S-Acylated and Behaves Like an Integral Membrane Protein

(2017) Macarena Rodriguez-Walker et al.   Scientific Reports

Complete Acid Ceramidase ablation prevents cancer-initiating cell formation in melanoma cells

(2017) Michele Lai et al.   Scientific Reports

Glucosylsphingosine is a key biomarker of Gaucher disease

(2016) Vagishwari Murugesan et al.   AMERICAN JOURNAL OF HEMATOLOGY

Neuronal sphingolipidoses: Membrane lipids and sphingolipid activator proteins regulate lysosomal sphingolipid catabolism

(2016) Konrad Sandhoff   BIOCHIMIE

Structural Mechanism for Cargo Recognition by the Retromer Complex

(2016) María Lucas et al.   CELL

NuclearDrosophilaCerS Schlank regulates lipid homeostasis via the homeodomain, independent of the lag1p motif

(2016) André Voelzmann et al.   FEBS LETTERS

Enzyme Activities of the Ceramide Synthases CERS2–6 Are Regulated by Phosphorylation in the C-terminal Region

(2016) Takayuki Sassa et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Ceramide Synthase 5 Is Essential to Maintain C16:0-Ceramide Pools and Contributes to the Development of Diet-induced Obesity

(2016) Dominic Gosejacob et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Localization of 1-deoxysphingolipids to mitochondria induces mitochondrial dysfunction

(2016) Irina Alecu et al.   JOURNAL OF LIPID RESEARCH

Gangliosides of the Vertebrate Nervous System

(2016) Ronald L. Schnaar   JOURNAL OF MOLECULAR BIOLOGY

Determining the Turnover of Glycosphingolipid Species by Stable-Isotope Tracer Lipidomics

(2016) Tore Skotland et al.   JOURNAL OF MOLECULAR BIOLOGY

The role of sphingolipids in neuronal plasticity of the brain

(2016) Sandro Sonnino et al.   JOURNAL OF NEUROCHEMISTRY

Glycans Confer Specificity to the Recognition of Ganglioside Receptors by Botulinum Neurotoxin A

(2016) Christoffer Hamark et al.   JOURNAL OF THE AMERICAN CHEMICAL SOCIETY

Sialic acids as link to Japanese scientists

(2016) Roland SCHAUER   PROCEEDINGS OF THE JAPAN ACADEMY SERIES B-PHYSICAL AND BIOLOGICAL SCIENCES

The enigma of ceramide synthase regulation in mammalian cells

(2016) Marthe-Susanna Wegner et al.   PROGRESS IN LIPID RESEARCH

Synthesis and degradation pathways, functions, and pathology of ceramides and epidermal acylceramides

(2016) Akio Kihara   PROGRESS IN LIPID RESEARCH

Distinct selectivity of gangliosides required for CD4 + T and CD8 + T cell activation

(2015) Jin-ichi Inokuchi et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR AND CELL BIOLOGY OF LIPIDS

Disialoganglioside GD2 as a therapeutic target for human diseases

(2015) Maya Suzuki et al.   EXPERT OPINION ON THERAPEUTIC TARGETS

Phosphatidic acid-mediated activation and translocation to the cell surface of sialidase NEU3, promoting signaling for cell migration

(2015) Kazuhiro Shiozaki et al.   FASEB JOURNAL

Ganglioside GM3 as a gatekeeper of obesity-associated insulin resistance: Evidence and mechanisms

(2015) Christopher Lipina et al.   FEBS LETTERS

GM3 and cancer

(2015) Sen-Itiroh Hakomori et al.   GLYCOCONJUGATE JOURNAL

Male meiotic cytokinesis requires ceramide synthase 3-dependent sphingolipids with unique membrane anchors

(2015) Mariona Rabionet et al.   HUMAN MOLECULAR GENETICS

Ganglioside GM3 is essential for the structural integrity and function of cochlear hair cells

(2015) M. Yoshikawa et al.   HUMAN MOLECULAR GENETICS

Membrane-spanning lipids for an uncompromised monitoring of membrane fusion and intermembrane lipid transfer

(2015) Günter Schwarzmann et al.   JOURNAL OF LIPID RESEARCH

Membrane lipids regulate ganglioside GM2 catabolism and GM2 activator protein activity

(2015) Susi Anheuser et al.   JOURNAL OF LIPID RESEARCH

Longitudinal changes in cerebellar and subcortical volumes in adult-onset Niemann–Pick disease type C patients treated with miglustat

(2015) Elizabeth A. Bowman et al.   JOURNAL OF NEUROLOGY

Stable or improved neurological manifestations during miglustat therapy in patients from the international disease registry for Niemann-Pick disease type C: an observational cohort study

(2015) Marc C Patterson et al.   Orphanet Journal of Rare Diseases

Elevation of 20-carbon long chain bases due to a mutation in serine palmitoyltransferase small subunit b results in neurodegeneration

(2015) Lihong Zhao et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Pathogenesis, emerging therapeutic targets and treatment in sialidosis

(2015) Alessandra D’Azzo et al.   Expert Opinion on Orphan Drugs

Metabolic and cellular bases of sphingolipidoses

(2015) Konrad Sandhoff   BIOCHEMICAL SOCIETY TRANSACTIONS

Impairment of ceramide synthesis causes a novel progressive myoclonus epilepsy

(2014) Nicola Vanni et al.   ANNALS OF NEUROLOGY

Ceramide synthase 4 deficiency in mice causes lipid alterations in sebum and results in alopecia

(2014) Philipp Ebel et al.   BIOCHEMICAL JOURNAL

Regulation of ceramide synthase 6 in a spontaneous experimental autoimmune encephalomyelitis model is sex dependent

(2014) Max Eberle et al.   BIOCHEMICAL PHARMACOLOGY

C16:0-Ceramide Signals Insulin Resistance

(2014) Timothy Hla et al.   Cell Metabolism

Simplifying complexity: genetically resculpting glycosphingolipid synthesis pathways in mice to reveal function

(2014) Maria Laura Allende et al.   GLYCOCONJUGATE JOURNAL

Expression of the ORMDLS, Modulators of Serine Palmitoyltransferase, Is Regulated by Sphingolipids in Mammalian Cells

(2014) Sita D. Gupta et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Complex lipid trafficking in Niemann-Pick disease type C

(2014) Marie T. Vanier   JOURNAL OF INHERITED METABOLIC DISEASE

Acid sphingomyelinase activity is regulated by membrane lipids and facilitates cholesterol transfer by NPC2

(2014) Vincent O. Oninla et al.   JOURNAL OF LIPID RESEARCH

Structural Basis for Substrate Specificity of Mammalian Neuraminidases

(2014) Victoria Smutova et al.   PLoS One

Lipid requirements for entry of protein toxins into cells

(2014) Kirsten Sandvig et al.   PROGRESS IN LIPID RESEARCH

Sphingolipid Metabolism and Interorganellar Transport: Localization of Sphingolipid Enzymes and Lipid Transfer Proteins

(2014) Toshiyuki Yamaji et al.   TRAFFIC

Ceramide synthesis in the epidermis

(2013) Mariona Rabionet et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR AND CELL BIOLOGY OF LIPIDS

Sphingolipids and lysosomal pathologies

(2013) Heike Schulze et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR AND CELL BIOLOGY OF LIPIDS

The role of sphingolipid metabolism in cutaneous permeabilitybarrier formation

(2013) Bernadette Breiden et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR AND CELL BIOLOGY OF LIPIDS

Mutations in B4GALNT1 (GM2 synthase) underlie a new disorder of ganglioside biosynthesis

(2013) Gaurav V. Harlalka et al.   BRAIN

 4GalT6 is involved in the synthesis of lactosylceramide with less intensity than  4GalT5

(2013) N. Tokuda et al.   GLYCOBIOLOGY

Expression machinery of GM4: the excess amounts of GM3/GM4S synthase (ST3GAL5) are necessary for GM4 synthesis in mammalian cells

(2013) Satoshi Uemura et al.   GLYCOCONJUGATE JOURNAL

A mutation in a ganglioside biosynthetic enzyme, ST3GAL5, results in salt & pepper syndrome, a neurocutaneous disorder with altered glycolipid and glycoprotein glycosylation

(2013) Luigi Boccuto et al.   HUMAN MOLECULAR GENETICS

Ganglioside GM1-mediated Transcytosis of Cholera Toxin Bypasses the Retrograde Pathway and Depends on the Structure of the Ceramide Domain

(2013) David E. Saslowsky et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Inactivation of Ceramide Synthase 6 in Mice Results in an Altered Sphingolipid Metabolism and Behavioral Abnormalities

(2013) Philipp Ebel et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Topological and Functional Characterization of the ssSPTs, Small Activating Subunits of Serine Palmitoyltransferase

(2013) Jeffrey M. Harmon et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Impaired Epidermal Ceramide Synthesis Causes Autosomal Recessive Congenital Ichthyosis and Reveals the Importance of Ceramide Acyl Chain Length

(2013) Katja-Martina Eckl et al.   JOURNAL OF INVESTIGATIVE DERMATOLOGY

Gangliosides and Gangliosidoses: Principles of Molecular and Metabolic Pathogenesis

(2013) K. Sandhoff et al.   JOURNAL OF NEUROSCIENCE

Lysosomal NEU1 deficiency affects amyloid precursor protein levels and amyloid-β secretion via deregulated lysosomal exocytosis

(2013) Ida Annunziata et al.   Nature Communications

Neuronal Expression of Glucosylceramide Synthase in Central Nervous System Regulates Body Weight and Energy Homeostasis

(2013) Viola Nordström et al.   PLOS BIOLOGY

Mutations in CERS3 Cause Autosomal Recessive Congenital Ichthyosis in Humans

(2013) Franz P. W. Radner et al.   PLoS Genetics

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

Construction of Conditional Acid Ceramidase Knockout Mice andin vivoEffects on Oocyte Development and Fertility

(2012) Efrat Eliyahu et al.   CELLULAR PHYSIOLOGY AND BIOCHEMISTRY

Lipid Sorting by Ceramide Structure from Plasma Membrane to ER for the Cholera Toxin Receptor Ganglioside GM1

(2012) Daniel J.-F. Chinnapen et al.   DEVELOPMENTAL CELL

Refractory epilepsy and mitochondrial dysfunction due to GM3 synthase deficiency

(2012) Konstantina Fragaki et al.   EUROPEAN JOURNAL OF HUMAN GENETICS

Glycosyltransferase complexes improve glycolipid synthesis

(2012) Waldo Spessott et al.   FEBS LETTERS

Mammalian sialidases: Physiological and pathological roles in cellular functions

(2012) T. Miyagi et al.   GLYCOBIOLOGY

Disialyl gangliosides enhance tumor phenotypes with differential modalities

(2012) Koichi Furukawa et al.   GLYCOCONJUGATE JOURNAL

Carbohydrate to carbohydrate interaction in development process and cancer progression

(2012) Kazuko Handa et al.   GLYCOCONJUGATE JOURNAL

Ablation of very long acyl chain sphingolipids causes hepatic insulin resistance in mice due to altered detergent-resistant membranes

(2012) Joo-Won Park et al.   HEPATOLOGY

Ablation of Neuronal Ceramide Synthase 1 in Mice Decreases Ganglioside Levels and Expression of Myelin-associated Glycoprotein in Oligodendrocytes

(2012) Christina Ginkel et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Glycosphingolipids Are Essential for Intestinal Endocytic Function

(2012) Richard Jennemann et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Mammalian ORMDL Proteins Mediate the Feedback Response in Ceramide Biosynthesis

(2012) Deanna L. Siow et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Formation of GM1 Ganglioside Clusters on the Lipid Membrane Containing Sphingomyeline and Cholesterol

(2012) Kenichi Mori et al.   JOURNAL OF PHYSICAL CHEMISTRY B

Essential Roles of Gangliosides in the Formation and Maintenance of Membrane Microdomains in Brain Tissues

(2012) Yuhsuke Ohmi et al.   NEUROCHEMICAL RESEARCH

My journey into the world of sphingolipids and sphingolipidoses

(2012) Konrad SANDHOFF   PROCEEDINGS OF THE JAPAN ACADEMY SERIES B-PHYSICAL AND BIOLOGICAL SCIENCES

Autophagy proteins in macroendocytic engulfment

(2012) Oliver Florey et al.   TRENDS IN CELL BIOLOGY

Loss of ceramide synthase 3 causes lethal skin barrier disruption

(2011) Richard Jennemann et al.   HUMAN MOLECULAR GENETICS

Metabolic pathways and intracellular trafficking of gangliosides

(2011) Jose Luis Daniotti et al.   IUBMB LIFE

New findings on nuclear gangliosides: overview on metabolism and function

(2011) Robert Ledeen et al.   JOURNAL OF NEUROCHEMISTRY

Cell surface sphingolipid glycohydrolases in neuronal differentiation and aging in culture

(2011) Massimo Aureli et al.   JOURNAL OF NEUROCHEMISTRY

Mice Lacking Major Brain Gangliosides Develop Parkinsonism

(2011) Gusheng Wu et al.   NEUROCHEMICAL RESEARCH

A Deficiency of Ceramide Biosynthesis Causes Cerebellar Purkinje Cell Neurodegeneration and Lipofuscin Accumulation

(2011) Lihong Zhao et al.   PLoS Genetics

Molecular mechanisms of pathogenesis in a glycosphingolipid and a glycoprotein storage disease

(2010) Alessandra d'Azzo et al.   BIOCHEMICAL SOCIETY TRANSACTIONS

β4-Galactosyltransferase-5 is a lactosylceramide synthase essential for mouse extra-embryonic development

(2010) Toshikazu Nishie et al.   GLYCOBIOLOGY

Hepatic glycosphingolipid deficiency and liver function in mice

(2010) Richard Jennemann et al.   HEPATOLOGY

A Critical Role for Ceramide Synthase 2 in Liver Homeostasis

(2010) Yael Pewzner-Jung et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Hereditary Sensory Neuropathy Type 1 Is Caused by the Accumulation of Two Neurotoxic Sphingolipids

(2010) Anke Penno et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Role of endosomal membrane lipids and NPC2 in cholesterol transfer and membrane fusion

(2010) Misbaudeen Abdul-Hammed et al.   JOURNAL OF LIPID RESEARCH

Orm family proteins mediate sphingolipid homeostasis

(2010) David K. Breslow et al.   NATURE

Molecular mechanism of multivesicular body biogenesis by ESCRT complexes

(2010) Thomas Wollert et al.   NATURE

Revitalizing membrane rafts: new tools and insights

(2010) Kai Simons et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

Biological Function of the Cellular Lipid BMP—BMP as a Key Activator for Cholesterol Sorting and Membrane Digestion

(2010) Hichem D. Gallala et al.   NEUROCHEMICAL RESEARCH

Orm1 and Orm2 are conserved endoplasmic reticulum membrane proteins regulating lipid homeostasis and protein quality control

(2010) S. Han et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Membrane microdomains and insulin resistance

(2009) Jin-ichi Inokuchi   FEBS LETTERS

Sphingolipid topology and the dynamic organization and function of membrane proteins

(2009) Gerrit van Meer et al.   FEBS LETTERS

Sphingolipids and gangliosides of the nervous system in membrane function and dysfunction

(2009) Elena Posse de Chaves et al.   FEBS LETTERS

Very long chain sphingolipids: Tissue expression, function and synthesis

(2009) Roger Sandhoff   FEBS LETTERS

Adult Ceramide Synthase 2 (CERS2)-deficient Mice Exhibit Myelin Sheath Defects, Cerebellar Degeneration, and Hepatocarcinomas

(2009) Silke Imgrund et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Zebrafish and Mouse α2,3-Sialyltransferases Responsible for Synthesizing GM4 Ganglioside

(2009) Shin-ichi Chisada et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

The SPTLC3 Subunit of Serine Palmitoyltransferase Generates Short Chain Sphingoid Bases

(2009) Thorsten Hornemann et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Developmental changes in glycolipids and synchronized expression of nutrient transporters in the mouse small intestine

(2009) Azusa Yoneshige et al.   JOURNAL OF NUTRITIONAL BIOCHEMISTRY

Mice lacking ganglioside GM3 synthase exhibit complete hearing loss due to selective degeneration of the organ of Corti

(2009) M. Yoshikawa et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Identification of small subunits of mammalian serine palmitoyltransferase that confer distinct acyl-CoA substrate specificities

(2009) G. Han et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Identification of gangliosides recognized by IgG anti-GalNAc-GD1a antibodies in bovine spinal motor neurons and motor nerves

(2008) Hiide Yoshino et al.   BRAIN RESEARCH

Membrane lipids: where they are and how they behave

(2008) Gerrit van Meer et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

Crystal Structures of Human Saposins C and D: Implications for Lipid Recognition and Membrane Interactions

(2008) Maxim Rossmann et al.   STRUCTURE

Functional role of glycosphingolipids and gangliosides in control of cell adhesion, motility, and growth, through glycosynaptic microdomains

(2007) Adriane Regina Todeschini et al.   BIOCHIMICA ET BIOPHYSICA ACTA-GENERAL SUBJECTS