Common and Uncommon Pathogenic Cascades in Lysosomal Storage Diseases

Lysosomal storage diseases as disorders of autophagy

(2014) Carmine Settembre et al.   Autophagy

LAMP-2: A control step for phagosome and autophagosome maturation

(2014) Paul Saftig et al.   Autophagy

Lipids on Trial: The Search for the Offending Metabolite in Niemann-Pick type C Disease

(2010) Emyr Lloyd-Evans et al.   TRAFFIC

Microglial Physiology: Unique Stimuli, Specialized Responses

(2009) Richard M. Ransohoff et al.   Annual Review of Immunology

Characterization of Gaucher disease bone marrow mesenchymal stromal cells reveals an altered inflammatory secretome

(2009) P. M. Campeau et al.   BLOOD

Mucopolysaccharidosis IIIB: Oxidative damage and cytotoxic cell involvement in the neuronal pathogenesis

(2009) Guglielmo R.D. Villani et al.   BRAIN RESEARCH

Effects of treatments on inflammatory and apoptotic markers in the CNS of mice with globoid cell leukodystrophy

(2009) Paola Luzi et al.   BRAIN RESEARCH

Alterations in Oxidative Markers in the Cerebellum and Peripheral Organs in MPS I Mice

(2009) Gustavo Kellermann Reolon et al.   CELLULAR AND MOLECULAR NEUROBIOLOGY

No evidence for activation of the unfolded protein response in neuronopathic models of Gaucher disease

(2009) T. Farfel-Becker et al.   HUMAN MOLECULAR GENETICS

Association of autophagy with cholesterol-accumulated compartments in Niemann-Pick disease type C cells

(2009) Seiya Ishibashi et al.   JOURNAL OF CLINICAL NEUROSCIENCE

GM1-Ganglioside Accumulation at the Mitochondria-Associated ER Membranes Links ER Stress to Ca2+-Dependent Mitochondrial Apoptosis

(2009) Renata Sano et al.   MOLECULAR CELL

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

(2009) David Smith et al.   NEUROBIOLOGY OF DISEASE

Enhanced autophagy and mitochondrial aberrations in murine GM1-gangliosidosis

(2008) Ayumi Takamura et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

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

Oxidative stress in NPC1 deficient cells: protective effect of allopregnanolone

(2008) Stefania Zampieri et al.   JOURNAL OF CELLULAR AND MOLECULAR MEDICINE

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

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

Globotriaosylceramide induces oxidative stress and up-regulates cell adhesion molecule expression in Fabry disease endothelial cells

(2008) Jin-Song Shen et al.   MOLECULAR GENETICS AND METABOLISM

Niemann-Pick disease type C1 is a sphingosine storage disease that causes deregulation of lysosomal calcium

(2008) Emyr Lloyd-Evans et al.   NATURE MEDICINE