BCM-95 and (2-hydroxypropyl)-β-cyclodextrin reverse autophagy dysfunction and deplete stored lipids in Sap C-deficient fibroblasts

Gaucher disease due to saposin C deficiency is an inherited lysosomal disease caused by rapidly degraded mutant proteins

(2014) Marialetizia Motta et al.   HUMAN MOLECULAR GENETICS

2-Hydroxypropyl-β-cyclodextrin Promotes Transcription Factor EB-mediated Activation of Autophagy

(2014) Wensi Song et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Curcumin Chemosensitizes 5-Fluorouracil Resistant MMR-Deficient Human Colon Cancer Cells in High Density Cultures

(2014) Mehdi Shakibaei et al.   PLoS One

Curcumin promotes exosomes/microvesicles secretion that attenuates lysosomal cholesterol traffic impairment

(2013) Alberto Canfrán-Duque et al.   MOLECULAR NUTRITION & FOOD RESEARCH

Efficacy and Safety of Curcumin in Major Depressive Disorder: A Randomized Controlled Trial

(2013) Jayesh Sanmukhani et al.   PHYTOTHERAPY RESEARCH

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

(2013) Sovan Sarkar et al.   Cell Reports

Reduced cathepsins B and D cause impaired autophagic degradation that can be almost completely restored by overexpression of these two proteases in Sap C-deficient fibroblasts

(2012) M. Tatti et al.   HUMAN MOLECULAR GENETICS

Normalization of Cholesterol Homeostasis by 2-Hydroxypropyl-β-cyclodextrin in Neurons and Glia from Niemann-Pick C1 (NPC1)-deficient Mice

(2012) Kyle B. Peake et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Cyclodextrin mediates rapid changes in lipid balance inNpc1−/−mice without carrying cholesterol through the bloodstream

(2012) Anna M. Taylor et al.   JOURNAL OF LIPID RESEARCH

Unesterified Cholesterol Accumulation in Late Endosomes/Lysosomes Causes Neurodegeneration and Is Prevented by Driving Cholesterol Export from This Compartment

(2011) A. Aqul et al.   JOURNAL OF NEUROSCIENCE

Gaucher disease due to saposin C deficiency, previously described as non-neuronopathic form — No positive effects after 2-years of miglustat therapy

(2011) Anna Tylki-Szymańska et al.   MOLECULAR GENETICS AND METABOLISM

Lysosomal positioning coordinates cellular nutrient responses

(2011) Viktor I. Korolchuk et al.   NATURE CELL BIOLOGY

Saposin C mutations in Gaucher disease patients resulting in lysosomal lipid accumulation, saposin C deficiency, but normal prosaposin processing and sorting

(2010) A. M. Vaccaro et al.   HUMAN MOLECULAR GENETICS

A mutation in the saposin C domain of the sphingolipid activator protein (Prosaposin) gene causes neurodegenerative disease in mice

(2010) Azusa Yoneshige et al.   JOURNAL OF NEUROSCIENCE RESEARCH

Endocytosis of beta-cyclodextrins is responsible for cholesterol reduction in Niemann-Pick type C mutant cells

(2010) A. I. Rosenbaum et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Specific saposin C deficiency: CNS impairment and acid  -glucosidase effects in the mouse

(2009) Y. Sun et al.   HUMAN MOLECULAR GENETICS

A pilot cross-over study to evaluate human oral bioavailability of BCM-95®CG (BiocurcumaxTM), a novel bioenhanced preparation of curcumin

(2009) B Antony et al.   INDIAN JOURNAL OF PHARMACEUTICAL SCIENCES

Chronic Cyclodextrin Treatment of Murine Niemann-Pick C Disease Ameliorates Neuronal Cholesterol and Glycosphingolipid Storage and Disease Progression

(2009) Cristin D. Davidson et al.   PLoS One

Reversal of defective lysosomal transport in NPC disease ameliorates liver dysfunction and neurodegeneration in the npc1-/- mouse

(2009) B. Liu et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

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

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