Altered Expression of Ganglioside Metabolizing Enzymes Results in GM3 Ganglioside Accumulation in Cerebellar Cells of a Mouse Model of Juvenile Neuronal Ceroid Lipofuscinosis

mTORC1-independent TFEB activation via Akt inhibition promotes cellular clearance in neurodegenerative storage diseases

(2017) Michela Palmieri et al.   Nature Communications

GM3 synthase deficiency due toST3GAL5variants in two Korean female siblings: Masquerading as Rett syndrome-like phenotype

(2016) Jin Sook Lee et al.   AMERICAN JOURNAL OF MEDICAL GENETICS PART A

Revisiting the neuronal localization and trafficking of CLN3 in juvenile neuronal ceroid lipofuscinosis

(2016) Sandra Oetjen et al.   JOURNAL OF NEUROCHEMISTRY

Finding the most appropriate mouse model of juvenile CLN3 (Batten) disease for therapeutic studies: the importance of genetic background and gender

(2015) A. D. Kovacs et al.   Disease Models & Mechanisms

Unbiased Cell-based Screening in a Neuronal Cell Model of Batten Disease Highlights an Interaction between Ca2+Homeostasis, Autophagy, and CLN3 Protein Function

(2015) Uma Chandrachud et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Gene-Wise Association of Variants in Four Lysosomal Storage Disorder Genes in Neuropathologically Confirmed Lewy Body Disease

(2015) Lorraine N. Clark et al.   PLoS One

Altered levels of α-synuclein and sphingolipids in Batten disease lymphoblast cells

(2014) Sunyang Kang et al.   GENE

Sialic Acids in the Brain: Gangliosides and Polysialic Acid in Nervous System Development, Stability, Disease, and Regeneration

(2014) Ronald L. Schnaar et al.   PHYSIOLOGICAL REVIEWS

FRET-Assisted Determination of CLN3 Membrane Topology

(2014) Ewa Ratajczak et al.   PLoS One

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

(2013) Gaurav V. Harlalka et al.   BRAIN

CLN3 Loss Disturbs Membrane Microdomain Properties and Protein Transport in Brain Endothelial Cells

(2013) L. Tecedor et al.   JOURNAL OF NEUROSCIENCE

Alterations in ROS Activity and Lysosomal pH Account for Distinct Patterns of Macroautophagy in LINCL and JNCL Fibroblasts

(2013) José Manuel Vidal-Donet et al.   PLoS One

The juvenile Batten disease protein, CLN3, and its role in regulating anterograde and retrograde post-Golgi trafficking

(2012) Susan L Cotman et al.   Clinical Lipidology

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

Ganglioside GM1 induces phosphorylation of mutant huntingtin and restores normal motor behavior in Huntington disease mice

(2012) A. Di Pardo et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Characterization of the CLEAR network reveals an integrated control of cellular clearance pathways

(2011) Michela Palmieri et al.   HUMAN MOLECULAR GENETICS

Metabolic pathways and intracellular trafficking of gangliosides

(2011) Jose Luis Daniotti et al.   IUBMB LIFE

Distinct Early Molecular Responses to Mutations Causing vLINCL and JNCL Presage ATP Synthase Subunit C Accumulation in Cerebellar Cells

(2011) Yi Cao et al.   PLoS One

Impaired Ganglioside Metabolism in Huntington's Disease and Neuroprotective Role of GM1

(2010) V. Maglione et al.   JOURNAL OF NEUROSCIENCE

GM1 ganglioside in Parkinson's disease: Results of a five year open study

(2010) Jay S. Schneider et al.   JOURNAL OF THE NEUROLOGICAL SCIENCES

Sialic acids as regulators of molecular and cellular interactions

(2009) Roland Schauer   CURRENT OPINION IN STRUCTURAL BIOLOGY

Brain gangliosides in axon-myelin stability and axon regeneration

(2009) Ronald L. Schnaar   FEBS LETTERS

Binding of laminin-1 to monosialoganglioside GM1 in lipid rafts is crucial for neurite outgrowth

(2009) N. Ichikawa et al.   JOURNAL OF CELL SCIENCE

A Gene Network Regulating Lysosomal Biogenesis and Function

(2009) Marco Sardiello et al.   SCIENCE

Examining the levels of ganglioside and cholesterol in cell membrane on attenuation the cytotoxicity of beta-amyloid peptide

(2008) Ming-Shen Lin et al.   COLLOIDS AND SURFACES B-BIOINTERFACES

The role of glycosphingolipid metabolism in the developing brain

(2008) Robert K. Yu et al.   JOURNAL OF LIPID RESEARCH

Elimination of GD3 synthase improves memory and reduces amyloid-β plaque load in transgenic mice

(2008) Alexandra Bernardo et al.   NEUROBIOLOGY OF AGING

CLN3p Impacts Galactosylceramide Transport, Raft Morphology, and Lipid Content

(2008) Elena Rusyn et al.   PEDIATRIC RESEARCH