Adaptations of Energy Metabolism Associated with Increased Levels of Mitochondrial Cholesterol in Niemann-Pick Type C1-deficient Cells

Cardioprotection by the TSPO ligand 4′-chlorodiazepam is associated with inhibition of mitochondrial accumulation of cholesterol at reperfusion

(2013) Stéphanie Paradis et al.   CARDIOVASCULAR RESEARCH

Overexpression of a key regulator of lipid homeostasis, Scap, promotes respiration in prostate cancer cells

(2013) Anika Vinayak Prabhu et al.   FEBS LETTERS

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

(2013) Rao Fu et al.   HUMAN MOLECULAR GENETICS

APP/PS1 mice overexpressing SREBP-2 exhibit combined Aβ accumulation and tau pathology underlying Alzheimer's disease

(2013) Elisabet Barbero-Camps et al.   HUMAN MOLECULAR GENETICS

ATP increases within the lumen of the endoplasmic reticulum upon intracellular Ca2+ release

(2013) Neelanjan Vishnu et al.   MOLECULAR BIOLOGY OF THE CELL

Pre-Symptomatic Activation of Antioxidant Responses and Alterations in Glucose and Pyruvate Metabolism in Niemann-Pick Type C1-Deficient Murine Brain

(2013) Barry E. Kennedy et al.   PLoS One

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

Niemann-Pick Type C2 protein contributes to the transport of endosomal cholesterol to mitochondria without interacting with NPC1

(2012) Barry E. Kennedy et al.   JOURNAL OF LIPID RESEARCH

Parallel labeling experiments with [1,2-13C]glucose and [U-13C]glutamine provide new insights into CHO cell metabolism

(2012) Woo Suk Ahn et al.   METABOLIC ENGINEERING

On a fully closed state of native human type-1 VDAC enriched in Nonidet P40

(2012) Friedrich P. Thinnes et al.   MOLECULAR GENETICS AND METABOLISM

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

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

Quantitative Proteomic Analysis of Niemann-Pick Disease, Type C1 Cerebellum Identifies Protein Biomarkers and Provides Pathological Insight

(2012) Stephanie M. Cologna et al.   PLoS One

Assessing mitochondrial dysfunction in cells

(2011) Martin D. Brand et al.   BIOCHEMICAL JOURNAL

Leucine Zipper EF Hand-containing Transmembrane Protein 1 (Letm1) and Uncoupling Proteins 2 and 3 (UCP2/3) Contribute to Two Distinct Mitochondrial Ca2+Uptake Pathways

(2011) Markus Waldeck-Weiermair et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

A sensitive and specific LC-MS/MS method for rapid diagnosis of Niemann-Pick C1 disease from human plasma

(2011) Xuntian Jiang et al.   JOURNAL OF LIPID RESEARCH

Metabolic flux analysis of CHO cells at growth and non-growth phases using isotopic tracers and mass spectrometry

(2011) Woo Suk Ahn et al.   METABOLIC ENGINEERING

Niemann-Pick type C 1 function requires lumenal domain residues that mediate cholesterol-dependent NPC2 binding

(2011) M. S. Deffieu et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Cholesterol and peroxidized cardiolipin in mitochondrial membrane properties, permeabilization and cell death

(2010) Joan Montero et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

Modulation of Plant Mitochondrial VDAC by Phytosterols

(2010) Lamia Mlayeh et al.   BIOPHYSICAL JOURNAL

Identification of Surface Residues on Niemann-Pick C2 Essential for Hydrophobic Handoff of Cholesterol to NPC1 in Lysosomes

(2010) Michael L. Wang et al.   Cell Metabolism

Oxidative stress in Niemann–Pick disease, type C

(2010) Rao Fu et al.   MOLECULAR GENETICS AND METABOLISM

Oxidative stress activates the c-Abl/p73 proapoptotic pathway in Niemann-Pick type C neurons

(2010) Andres Klein et al.   NEUROBIOLOGY OF DISEASE

Mild oxidative stress activates Nrf2 in astrocytes, which contributes to neuroprotective ischemic preconditioning

(2010) K. F. Bell et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Niemann–Pick C2 (NPC2) and intracellular cholesterol trafficking

(2009) Judith Storch et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR AND CELL BIOLOGY OF LIPIDS

Mitochondria, cholesterol and amyloid β peptide: a dangerous trio in Alzheimer disease

(2009) Anna Colell et al.   JOURNAL OF BIOENERGETICS AND BIOMEMBRANES

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

Visualization of ATP levels inside single living cells with fluorescence resonance energy transfer-based genetically encoded indicators

(2009) H. Imamura et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Mitochondrial Cholesterol Contributes to Chemotherapy Resistance in Hepatocellular Carcinoma

(2008) J. Montero et al.   CANCER RESEARCH

Cholesterol interaction with the related steroidogenic acute regulatory lipid-transfer (START) domains of StAR (STARD1) and MLN64 (STARD3)

(2008) Julian Reitz et al.   FEBS Journal

Regulation of hexokinase binding to VDAC

(2008) John G. Pastorino et al.   JOURNAL OF BIOENERGETICS AND BIOMEMBRANES

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

Solution Structure of the Integral Human Membrane Protein VDAC-1 in Detergent Micelles

(2008) S. Hiller et al.   SCIENCE