Acat1/Soat1 knockout extends the mutant Npc1 mouse lifespan and ameliorates functional deficiencies in multiple organelles of mutant cells

Improved systemic AAV gene therapy with a neurotrophic capsid in Niemann–Pick disease type C1 mice

(2021) Cristin D Davidson et al.   Life Science Alliance

Facile method to incorporate high-affinity ACAT/SOAT1 inhibitor F12511 into stealth liposome-based nanoparticle and demonstration of its efficacy in blocking cholesteryl ester biosynthesis without overt toxicity in neuronal cell culture

(2021) Adrianna L. De La Torre et al.   JOURNAL OF NEUROSCIENCE METHODS

NPC intracellular cholesterol transporter 1 (NPC1)-mediated cholesterol export from lysosomes

(2019) Suzanne R. Pfeffer   JOURNAL OF BIOLOGICAL CHEMISTRY

ER–lysosome contacts enable cholesterol sensing by mTORC1 and drive aberrant growth signalling in Niemann–Pick type C

(2019) Chun-Yan Lim et al.   NATURE CELL BIOLOGY

NPC1 regulates ER contacts with endocytic organelles to mediate cholesterol egress

(2019) D. Höglinger et al.   Nature Communications

Miglustat in Niemann-Pick disease type C patients: a review

(2018) Mercè Pineda et al.   Orphanet Journal of Rare Diseases

Structure of the Golgi apparatus is not influenced by a GAG deletion mutation in the dystonia-associated gene Tor1a

(2018) Sara B. Mitchell et al.   PLoS One

Lipids regulate the hydrolysis of membrane bound glucosylceramide by lysosomal β-glucocerebrosidase

(2017) Misbaudeen Abdul-Hammed et al.   JOURNAL OF LIPID RESEARCH

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

Oxysterol-Binding Protein-Related Protein 1L Regulates Cholesterol Egress from the Endo-Lysosomal System

(2017) Kexin Zhao et al.   Cell Reports

Cholesterol Transport through Lysosome-Peroxisome Membrane Contacts

(2015) Bei-Bei Chu et al.   CELL

ACAT1/SOAT1 as a therapeutic target for Alzheimer's disease

(2015) Yohei Shibuya et al.   Future Medicinal Chemistry

Deficiency in the Lipid Exporter ABCA1 Impairs Retrograde Sterol Movement and Disrupts Sterol Sensing at the Endoplasmic Reticulum

(2015) Yoshio Yamauchi et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Cholesterol Regulates Syntaxin 6 Trafficking at trans-Golgi Network Endosomal Boundaries

(2014) Meritxell Reverter et al.   Cell Reports

Three pools of plasma membrane cholesterol and their relation to cholesterol homeostasis

(2014) Akash Das et al.   eLife

MAM (mitochondria-associated membranes) in mammalian cells: Lipids and beyond

(2013) Jean E. Vance   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR AND CELL BIOLOGY OF LIPIDS

A novel alkyne cholesterol to trace cellular cholesterol metabolism and localization

(2013) Kristina Hofmann et al.   JOURNAL OF LIPID RESEARCH

Proteome-wide mapping of cholesterol-interacting proteins in mammalian cells

(2013) Jonathan J Hulce et al.   NATURE METHODS

Visualization of Sterol-Rich Membrane Domains with Fluorescently-Labeled Theonellamides

(2013) Shinichi Nishimura et al.   PLoS One

Upregulated function of mitochondria-associated ER membranes in Alzheimer disease

(2012) Estela Area-Gomez et al.   EMBO JOURNAL

Calpain-mediated ABCA1 degradation: Post-translational regulation of ABCA1 for HDL biogenesis

(2011) Shinji Yokoyama et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR AND CELL BIOLOGY OF LIPIDS

A novel mouse model of Niemann–Pick type C disease carrying a D1005G-Npc1 mutation comparable to commonly observed human mutations

(2011) Robert A. Maue et al.   HUMAN MOLECULAR GENETICS

A role for oxysterol-binding protein–related protein 5 in endosomal cholesterol trafficking

(2011) Ximing Du et al.   JOURNAL OF CELL BIOLOGY

STARD4 abundance regulates sterol transport and sensing

(2011) Bruno Mesmin et al.   MOLECULAR BIOLOGY OF THE CELL

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

Selective Fluorescence Labeling of Lipids in Living Cells

(2009) Anne B. Neef et al.   ANGEWANDTE CHEMIE-INTERNATIONAL EDITION

MLN64 mediates egress of cholesterol from endosomes to mitochondria in the absence of functional Niemann-Pick Type C1 protein

(2009) Mark Charman et al.   JOURNAL OF LIPID RESEARCH

Mitochondrial Cholesterol Loading Exacerbates Amyloid   Peptide-Induced Inflammation and Neurotoxicity

(2009) A. Fernandez et al.   JOURNAL OF NEUROSCIENCE

Metabolic labeling and direct imaging of choline phospholipids in vivo

(2009) C. Y. Jao et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Cyclodextrin overcomes deficient lysosome-to-endoplasmic reticulum transport of cholesterol in Niemann-Pick type C cells

(2009) L. Abi-Mosleh et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Cathepsin D—Many functions of one aspartic protease

(2008) Petr Benes et al.   CRITICAL REVIEWS IN ONCOLOGY HEMATOLOGY

ACAT as a Drug Target for Alzheimer’s Disease

(2008) Henri J. Huttunen et al.   Neurodegenerative Diseases

Transport of LDL-derived cholesterol from the NPC1 compartment to the ER involves the trans-Golgi network and the SNARE protein complex

(2008) Y. Urano et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA