Genetic and pharmacological evidence implicates cathepsins in Niemann-Pick C cerebellar degeneration

Ferritin-stimulated lipid peroxidation, lysosomal leak, and macroautophagy promote lysosomal “metastability” in primary hepatocytes determining in vitro cell survival

(2015) Margit A. Krenn et al.   FREE RADICAL BIOLOGY AND MEDICINE

A Murine Niemann-Pick C1 I1061T Knock-In Model Recapitulates the Pathological Features of the Most Prevalent Human Disease Allele

(2015) M. Praggastis et al.   JOURNAL OF NEUROSCIENCE

Identification of NPC1 as the target of U18666A, an inhibitor of lysosomal cholesterol export and Ebola infection

(2015) Feiran Lu et al.   eLife

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

Inhibition of cysteine cathepsin B and L activation in astrocytes contributes to neuroprotection against cerebral ischemia via blocking the tBid-mitochondrial apoptotic signaling pathway

(2014) Min Xu et al.   GLIA

A Role for Stefin B (Cystatin B) in Inflammation and Endotoxemia

(2014) Katarina Maher et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Efficacy of N-acetylcysteine in phenotypic suppression of mouse models of Niemann–Pick disease, type C1

(2013) Rao Fu et al.   HUMAN MOLECULAR GENETICS

The Cysteine Protease Cathepsin B Is a Key Drug Target and Cysteine Protease Inhibitors Are Potential Therapeutics for Traumatic Brain Injury

(2013) Gregory R. Hook et al.   JOURNAL OF NEUROTRAUMA

Cysteine Cathepsins in Neurological Disorders

(2013) Anja Pišlar et al.   MOLECULAR NEUROBIOLOGY

Signals from the lysosome: a control centre for cellular clearance and energy metabolism

(2013) Carmine Settembre et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

Stefin B deficiency reduces tumor growth via sensitization of tumor cells to oxidative stress in a breast cancer model

(2013) M Butinar et al.   ONCOGENE

Impaired Autophagy in the Lipid-Storage Disorder Niemann-Pick Type C1 Disease

(2013) Sovan Sarkar et al.   Cell Reports

Autophagy in lysosomal storage disorders

(2012) Andrew P. Lieberman et al.   Autophagy

Autophagic dysfunction in a lysosomal storage disorder due to impaired proteolysis

(2012) Matthew J. Elrick et al.   Autophagy

Disruption and therapeutic rescue of autophagy in a human neuronal model of Niemann Pick type C1

(2012) M. Paulina Ordonez et al.   HUMAN MOLECULAR GENETICS

Ryanodine receptor antagonists adapt NPC1 proteostasis to ameliorate lipid storage in Niemann–Pick type C disease fibroblasts

(2012) Ting Yu et al.   HUMAN MOLECULAR GENETICS

Impaired proteolysis underlies autophagic dysfunction in Niemann–Pick type C disease

(2012) Matthew J. Elrick et al.   HUMAN MOLECULAR GENETICS

Oxidative Stress: A Pathogenic Mechanism for Niemann-Pick Type C Disease

(2012) Mary Carmen Vázquez et al.   Oxidative Medicine and Cellular Longevity

Clarifying lysosomal storage diseases

(2011) Mark L. Schultz et al.   TRENDS IN NEUROSCIENCES

Reversal of autophagy dysfunction in the TgCRND8 mouse model of Alzheimer's disease ameliorates amyloid pathologies and memory deficits

(2010) Dun-Sheng Yang et al.   BRAIN

Combating apoptosis and multidrug resistant cancers by targeting lysosomes

(2010) Line Groth-Pedersen et al.   CANCER LETTERS

Cholesterol Oxidation Products Are Sensitive and Specific Blood-Based Biomarkers for Niemann-Pick C1 Disease

(2010) F. D. Porter et al.   Science Translational Medicine

Increased Activity and Altered Subcellular Distribution of Lysosomal Enzymes Determine Neuronal Vulnerability in Niemann-Pick Type C1-Deficient Mice

(2009) Asha Amritraj et al.   AMERICAN JOURNAL OF PATHOLOGY

VEGF-A Induces Angiogenesis by Perturbing the Cathepsin-Cysteine Protease Inhibitor Balance in Venules, Causing Basement Membrane Degradation and Mother Vessel Formation

(2009) S.-H. Chang et al.   CANCER RESEARCH

Structure of N-Terminal Domain of NPC1 Reveals Distinct Subdomains for Binding and Transfer of Cholesterol

(2009) Hyock Joo Kwon et al.   CELL

Conditional Niemann-Pick C mice demonstrate cell autonomous Purkinje cell neurodegeneration

(2009) Matthew J. Elrick et al.   HUMAN MOLECULAR GENETICS

Cystatin B Deficiency Sensitizes Neurons to Oxidative Stress in Progressive Myoclonus Epilepsy, EPM1

(2009) M. K. Lehtinen et al.   JOURNAL OF NEUROSCIENCE

Inhibitors of Cathepsin B Improve Memory and Reduce β-Amyloid in Transgenic Alzheimer Disease Mice Expressing the Wild-type, but Not the Swedish Mutant, β-Secretase Site of the Amyloid Precursor Protein

(2008) Vivian Y. H. Hook et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Oxidative stress in NPC1 deficient cells: protective effect of allopregnanolone

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

NPC2 facilitates bidirectional transfer of cholesterol between NPC1 and lipid bilayers, a step in cholesterol egress from lysosomes

(2008) R. E. Infante et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Apoptosis: controlled demolition at the cellular level

(2007) Rebecca C. Taylor et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY