Neuroinflammatory paradigms in lysosomal storage diseases

Astrogliopathology in neurological, neurodevelopmental and psychiatric disorders

(2016) Alexei Verkhratsky et al.   NEUROBIOLOGY OF DISEASE

Astrocytes and lysosomal storage diseases

(2016) K.V. Rama Rao et al.   NEUROSCIENCE

DAMPs and neurodegeneration

(2015) John Thundyil et al.   AGEING RESEARCH REVIEWS

Innate immune responses in the brain of sphingolipid lysosomal storage diseases

(2015) Einat B. Vitner et al.   BIOLOGICAL CHEMISTRY

Impaired migration capacity in monocytes derived from patients with Gaucher disease

(2015) Noam Bettman et al.   BLOOD CELLS MOLECULES AND DISEASES

Mechanism-Based Combination Treatment Dramatically Increases Therapeutic Efficacy in Murine Globoid Cell Leukodystrophy

(2015) J. A. Hawkins-Salsbury et al.   JOURNAL OF NEUROSCIENCE

Neuroinflammation in Alzheimer's disease

(2015) Michael T Heneka et al.   LANCET NEUROLOGY

Biomarkers of central nervous system inflammation in infantile and juvenile gangliosidoses

(2015) Jeanine R. Jarnes Utz et al.   MOLECULAR GENETICS AND METABOLISM

Neuromodulatory properties of inflammatory cytokines and their impact on neuronal excitability

(2015) Annamaria Vezzani et al.   NEUROPHARMACOLOGY

Intracisternal cyclodextrin prevents cerebellar dysfunction and Purkinje cell death in feline Niemann-Pick type C1 disease

(2015) Charles H. Vite et al.   Science Translational Medicine

Gaucher Disease and Its Treatment Options

(2014) Lunawati L. Bennett et al.   ANNALS OF PHARMACOTHERAPY

Secondary biochemical and morphological consequences in lysosomal storage diseases

(2014) J. Alroy et al.   BIOCHEMISTRY-MOSCOW

Type II NKT-TFH cells against Gaucher lipids regulate B-cell immunity and inflammation

(2014) S. Nair et al.   BLOOD

Gene therapy for neurologic manifestations of mucopolysaccharidoses

(2014) Daniel A Wolf et al.   Expert Opinion on Drug Delivery

Plasma Signature of Neurological Disease in the Monogenetic Disorder Niemann-Pick Type C

(2014) Md. Suhail Alam et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

PERK-Dependent Activation of JAK1 and STAT3 Contributes to Endoplasmic Reticulum Stress-Induced Inflammation

(2014) G. P. Meares et al.   MOLECULAR AND CELLULAR BIOLOGY

Progressive Parkinsonism by acute dysfunction of excitatory amino acid transporters in the rat substantia nigra

(2014) Maxime Assous et al.   NEUROBIOLOGY OF DISEASE

Improved neuroprotection using miglustat, curcumin and ibuprofen as a triple combination therapy in Niemann–Pick disease type C1 mice

(2014) Ian M. Williams et al.   NEUROBIOLOGY OF DISEASE

Why are Astrocytes Important?

(2014) Alexei Verkhratsky et al.   NEUROCHEMICAL RESEARCH

Evidence for Aberrant Astrocyte Hemichannel Activity in Juvenile Neuronal Ceroid Lipofuscinosis (JNCL)

(2014) Maria Burkovetskaya et al.   PLoS One

Fingolimod for the treatment of neurological diseases—state of play and future perspectives

(2014) Robert Brunkhorst et al.   Frontiers in Cellular Neuroscience

How Do Astrocytes Participate in Neural Plasticity?

(2014) Philip G. Haydon et al.   Cold Spring Harbor Perspectives in Biology

Calcium dysregulation and neuroinflammation: Discrete and integrated mechanisms for age-related synaptic dysfunction

(2013) Diana M. Sama et al.   AGEING RESEARCH REVIEWS

Astrocytes and Glutamate Homoeostasis in Alzheimer's Disease: A Decrease in Glutamine Synthetase, But Not in Glutamate Transporter-1, in the Prefrontal Cortex

(2013) Magdalena Kulijewicz-Nawrot et al.   ASN Neuro

Pathogenesis and therapies for infantile neuronal ceroid lipofuscinosis (infantile CLN1 disease)

(2013) Jacqueline A. Hawkins-Salsbury et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

NCL disease mechanisms

(2013) David N. Palmer et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Cell biology and function of neuronal ceroid lipofuscinosis-related proteins

(2013) Katrin Kollmann et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Inflammation in neurodegenerative diseases - an update

(2013) Sandra Amor et al.   IMMUNOLOGY

Batten Disease

(2013) Sarah-Bianca Dolisca et al.   JOURNAL OF CHILD NEUROLOGY

Mucopolysaccharide diseases: A complex interplay between neuroinflammation, microglial activation and adaptive immunity

(2013) Louise D. Archer et al.   JOURNAL OF INHERITED METABOLIC DISEASE

Microglia in juvenile neuronal ceroid lipofuscinosis are primed toward a pro-inflammatory phenotype

(2013) Juan Xiong et al.   JOURNAL OF NEUROCHEMISTRY

Fabry disease peripheral blood immune cells release inflammatory cytokines: Role of globotriaosylceramide

(2013) Pablo N. De Francesco et al.   MOLECULAR GENETICS AND METABOLISM

Lysosomal storage diseases—the horizon expands

(2013) Rose-Mary Naaman Boustany   Nature Reviews Neurology

Neuroinflammation: The role and consequences

(2013) Monty Lyman et al.   NEUROSCIENCE RESEARCH

Molecular epidemiology of childhood neuronal ceroid-lipofuscinosis in Italy

(2013) Filippo Maria Santorelli et al.   Orphanet Journal of Rare Diseases

The lysosome: from waste bag to potential therapeutic target

(2013) Hanna Appelqvist et al.   Journal of Molecular Cell Biology

Synergistic effects of central nervous system-directed gene therapy and bone marrow transplantation in the murine model of infantile neuronal ceroid lipofuscinosis

(2012) Shannon L. Macauley et al.   ANNALS OF NEUROLOGY

Human pathology in NCL

(2012) Glenn W. Anderson et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Contribution of brain inflammation to neuronal cell death in neuronopathic forms of Gaucher's disease

(2012) Einat B. Vitner et al.   BRAIN

Ceramide function in the brain: when a slight tilt is enough

(2012) Chiara Mencarelli et al.   CELLULAR AND MOLECULAR LIFE SCIENCES

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

Lysosomal storage disorders: The cellular impact of lysosomal dysfunction

(2012) Frances M. Platt et al.   JOURNAL OF CELL BIOLOGY

Mucopolysaccharidosis type I: current knowledge on its pathophysiological mechanisms

(2012) Derbis Campos et al.   METABOLIC BRAIN DISEASE

Bone marrow transplantation increases efficacy of central nervous system-directed enzyme replacement therapy in the murine model of globoid cell leukodystrophy

(2012) Elizabeth Y. Qin et al.   MOLECULAR GENETICS AND METABOLISM

Neuropathology in Mouse Models of Mucopolysaccharidosis Type I, IIIA and IIIB

(2012) Fiona L. Wilkinson et al.   PLoS One

Niemann-Pick Type C1 deficiency in microglia does not cause neuron death in vitro

(2011) Kyle B. Peake et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Toll-like receptors in health and disease in the brain: mechanisms and therapeutic potential

(2011) Mark L. Hanke et al.   CLINICAL SCIENCE

The Batten disease gene CLN3 is required for the response to oxidative stress

(2011) Richard I. Tuxworth et al.   HUMAN MOLECULAR GENETICS

Combination therapies for lysosomal storage disease: is the whole greater than the sum of its parts?

(2011) J. A. Hawkins-Salsbury et al.   HUMAN MOLECULAR GENETICS

Spatial and temporal correlation between neuron loss and neuroinflammation in a mouse model of neuronopathic Gaucher disease

(2011) T. Farfel-Becker et al.   HUMAN MOLECULAR GENETICS

Unfolded proteins and endoplasmic reticulum stress in neurodegenerative disorders

(2011) Karen M. Doyle et al.   JOURNAL OF CELLULAR AND MOLECULAR MEDICINE

Regulation of the NPC2 protein-mediated cholesterol trafficking by membrane lipids

(2011) Hichem D. Gallala et al.   JOURNAL OF NEUROCHEMISTRY

Neuroinflammation Leads to Region-Dependent Alterations in Astrocyte Gap Junction Communication and Hemichannel Activity

(2011) N. Karpuk et al.   JOURNAL OF NEUROSCIENCE

Amyloid  -Induced Death in Neurons Involves Glial and Neuronal Hemichannels

(2011) J. A. Orellana et al.   JOURNAL OF NEUROSCIENCE

The cellular pathology of lysosomal diseases

(2011) Timothy M Cox et al.   JOURNAL OF PATHOLOGY

Neuroinflammatory and oxidative stress phenomena in MPS IIIA mouse model: The positive effect of long-term aspirin treatment

(2011) Audrey Arfi et al.   MOLECULAR GENETICS AND METABOLISM

Early glial activation, synaptic changes and axonal pathology in the thalamocortical system of Niemann–Pick type C1 mice

(2011) Sarah N.R. Pressey et al.   NEUROBIOLOGY OF DISEASE

Altered sensitivity of cerebellar granule cells to glutamate receptor overactivation in the Cln3Δex7/8-knock-in mouse model of juvenile neuronal ceroid lipofuscinosis

(2011) Rozzy Finn et al.   NEUROCHEMISTRY INTERNATIONAL

Calcium Dysregulation and Homeostasis of Neural Calcium in the Molecular Mechanisms of Neurodegenerative Diseases Provide Multiple Targets for Neuroprotection

(2010) Gregor Zündorf et al.   ANTIOXIDANTS & REDOX SIGNALING

Identification of Surface Residues on Niemann-Pick C2 Essential for Hydrophobic Handoff of Cholesterol to NPC1 in Lysosomes

(2010) Michael L. Wang et al.   Cell Metabolism

Common and Uncommon Pathogenic Cascades in Lysosomal Storage Diseases

(2010) Einat B. Vitner et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

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

Niemann-Pick type C disease: molecular mechanisms and potential therapeutic approaches

(2010) Anton I. Rosenbaum et al.   JOURNAL OF NEUROCHEMISTRY

Niemann-Pick disease type C

(2010) Marie T Vanier   Orphanet Journal of Rare Diseases

Unilaterally and rapidly progressing white matter lesion and elevated cytokines in a patient with Tay–Sachs disease

(2009) Tomomi Hayase et al.   BRAIN & DEVELOPMENT

Sphingolipids in inflammation: pathological implications and potential therapeutic targets

(2009) Graeme F. Nixon   BRITISH JOURNAL OF PHARMACOLOGY

ER stress in Alzheimer's disease: a novel neuronal trigger for inflammation and Alzheimer's pathology

(2009) Antero Salminen et al.   Journal of Neuroinflammation

AIM2 activates the inflammasome and cell death in response to cytoplasmic DNA

(2009) Teresa Fernandes-Alnemri et al.   NATURE

Pyroptosis: host cell death and inflammation

(2009) Tessa Bergsbaken et al.   NATURE REVIEWS MICROBIOLOGY

Beneficial effects of anti-inflammatory therapy in a mouse model of Niemann-Pick disease type C1

(2009) David Smith et al.   NEUROBIOLOGY OF DISEASE

Lipid Mediators in the Neural Cell Nucleus: Their Metabolism, Signaling, and Association with Neurological Disorders

(2009) Akhlaq A. Farooqui   NEUROSCIENTIST

Neuronal ceroid lipofuscinoses

(2008) Anu Jalanko et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

Lysosomal disorders: From storage to cellular damage

(2008) Andrea Ballabio et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

Disease-Specific Induced Pluripotent Stem Cells

(2008) In-Hyun Park et al.   CELL

Innate and adaptive immune activation in the brain of MPS IIIB mouse model

(2008) Julianne DiRosario et al.   JOURNAL OF NEUROSCIENCE RESEARCH