The susceptibility of cochlear outer hair cells to cyclodextrin is not related to their electromotile activity

2-Hydroxypropyl-β-cyclodextrins and the Blood-Brain Barrier: Considerations for Niemann-Pick Disease Type C1

(2018) Pericles Calias   CURRENT PHARMACEUTICAL DESIGN

Prestin Contributes to Membrane Compartmentalization and Is Required for Normal Innervation of Outer Hair Cells

(2018) Satoe Takahashi et al.   Frontiers in Cellular Neuroscience

Intrathecal 2-hydroxypropyl-β-cyclodextrin decreases neurological disease progression in Niemann-Pick disease, type C1: a non-randomised, open-label, phase 1–2 trial

(2017) Daniel S Ory et al.   LANCET

Cyclodextrins as Emerging Therapeutic Tools in the Treatment of Cholesterol-Associated Vascular and Neurodegenerative Diseases

(2016) Caroline Coisne et al.   MOLECULES

Lysosomal membrane glycoproteins bind cholesterol and contribute to lysosomal cholesterol export

(2016) Jian Li et al.   eLife

Efficacy and ototoxicity of different cyclodextrins in Niemann-Pick C disease

(2016) Cristin D. Davidson et al.   Annals of Clinical and Translational Neurology

Susceptibility of outer hair cells to cholesterol chelator 2-hydroxypropyl-β-cyclodextrine is prestin-dependent

(2016) Satoe Takahashi et al.   Scientific Reports

Efficacy of 2-Hydroxypropyl-β-cyclodextrin in Niemann–Pick Disease Type C Model Mice and Its Pharmacokinetic Analysis in a Patient with the Disease

(2015) Yuta Tanaka et al.   BIOLOGICAL & PHARMACEUTICAL BULLETIN

Dynamics of cochlear synaptopathy after acoustic overexposure

(2015) Leslie D. Liberman et al.   JARO-JOURNAL OF THE ASSOCIATION FOR RESEARCH IN OTOLARYNGOLOGY

Hearing Loss and Otopathology Following Systemic and Intracerebroventricular Delivery of 2-Hydroxypropyl-Beta-Cyclodextrin

(2015) Scott Cronin et al.   JARO-JOURNAL OF THE ASSOCIATION FOR RESEARCH IN OTOLARYNGOLOGY

Unfolding and refolding of a protein by cholesterol and cyclodextrin: a single molecule study

(2015) Shirsendu Ghosh et al.   PHYSICAL CHEMISTRY CHEMICAL PHYSICS

Prestin-Dependence of Outer Hair Cell Survival and Partial Rescue of Outer Hair Cell Loss in PrestinV499G/Y501H Knockin Mice

(2015) Mary Ann Cheatham et al.   PLoS One

Collaborative Development of 2-Hydroxypropyl-β-Cyclodextrin for the Treatment of Niemann-Pick Type C1 Disease

(2014) Elizabeth Ottinger et al.   CURRENT TOPICS IN MEDICINAL CHEMISTRY

Hearing Loss is an Early Consequence of Npc1 Gene Deletion in the Mouse Model of Niemann–Pick Disease, Type C

(2014) Kelly A. King et al.   JARO-JOURNAL OF THE ASSOCIATION FOR RESEARCH IN OTOLARYNGOLOGY

Niemann-Pick C disease and mobilization of lysosomal cholesterol by cyclodextrin

(2014) Jean E. Vance et al.   JOURNAL OF LIPID RESEARCH

Loss of the Tectorial Membrane Protein CEACAM16 Enhances Spontaneous, Stimulus-Frequency, and Transiently Evoked Otoacoustic Emissions

(2014) M. A. Cheatham et al.   JOURNAL OF NEUROSCIENCE

Lysosomal-specific Cholesterol Reduction by Biocleavable Polyrotaxanes for Ameliorating Niemann-Pick Type C Disease

(2014) Atsushi Tamura et al.   Scientific Reports

Auditory Phenotype of Niemann-Pick Disease, Type C1

(2013) Kelly A. King et al.   EAR AND HEARING

Membrane Cholesterol Strongly Influences Confined Diffusion of Prestin

(2012) R.I. Kamar et al.   BIOPHYSICAL JOURNAL

Therapeutic potential of cyclodextrins in the treatment of Niemann–Pick type C disease

(2012) Benny Liu   Clinical Lipidology

Microarray expression analysis and identification of serum biomarkers for Niemann–Pick disease, type C1

(2012) Celine V.M. Cluzeau et al.   HUMAN MOLECULAR GENETICS

The V499G/Y501H Mutation Impairs Fast Motor Kinetics of Prestin and Has Significance for Defining Functional Independence of Individual Prestin Subunits

(2012) Kazuaki Homma 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

Hearing Loss and Hair Cell Death in Mice Given the Cholesterol-Chelating Agent Hydroxypropyl-β-Cyclodextrin

(2012) Mark A. Crumling et al.   PLoS One

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

Prestin forms oligomer with four mechanically independent subunits

(2010) Xiang Wang et al.   BRAIN RESEARCH

Atomic force microscopy imaging of the structure of the motor protein prestin reconstituted into an artificial lipid bilayer

(2010) Shun Kumano et al.   FEBS LETTERS

Evidence That Prestin Has at Least Two Voltage-dependent Steps

(2010) Kazuaki Homma et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

2-Hydroxypropyl-β-Cyclodextrin Raises Hearing Threshold in Normal Cats and in Cats With Niemann-Pick Type C Disease

(2010) Sarah Ward et al.   PEDIATRIC 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

Glycosylation Regulates Prestin Cellular Activity

(2009) Lavanya Rajagopalan et al.   JARO-JOURNAL OF THE ASSOCIATION FOR RESEARCH IN OTOLARYNGOLOGY

Cyclodextrin overcomes the transport defect in nearly every organ of NPC1 mice leading to excretion of sequestered cholesterol as bile acid

(2009) Benny Liu et al.   JOURNAL OF LIPID RESEARCH

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

(2009) David Smith et al.   NEUROBIOLOGY OF DISEASE

Prestin up-regulation in chronic salicylate (aspirin) administration: An implication of functional dependence of prestin expression

(2008) N. Yu et al.   CELLULAR AND MOLECULAR LIFE SCIENCES

 II- V spectrin bridges the plasma membrane and cortical lattice in the lateral wall of the auditory outer hair cells

(2008) K. Legendre et al.   JOURNAL OF CELL SCIENCE

Prestin-Based Outer Hair Cell Motility Is Necessary for Mammalian Cochlear Amplification

(2008) Peter Dallos et al.   NEURON