Lipid–Protein Interactions in Niemann–Pick Type C Disease: Insights from Molecular Modeling

Structural basis for PtdInsP2-mediated human TRPML1 regulation

(2018) Michael Fine et al.   Nature Communications

Identification of a feedback loop involving β-glucosidase 2 and its product sphingosine sheds light on the molecular mechanisms in Gaucher disease

(2017) Sophie Schonauer et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

A voltage-dependent K+channel in the lysosome is required for refilling lysosomal Ca2+stores

(2017) Wuyang Wang et al.   JOURNAL OF CELL BIOLOGY

The dual function of the polybasic juxtamembrane region of syntaxin 1A in clamping spontaneous release and stimulating Ca2+-triggered release in neuroendocrine cells

(2017) Dafna Singer-Lahat et al.   JOURNAL OF NEUROSCIENCE

Human TRPML1 channel structures in open and closed conformations

(2017) Philip Schmiege et al.   NATURE

Cryo-electron microscopy structure of the lysosomal calcium-permeable channel TRPML3

(2017) Marscha Hirschi et al.   NATURE

Structural basis of dual Ca2+/pH regulation of the endolysosomal TRPML1 channel

(2017) Minghui Li et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

3.3 Å structure of Niemann–Pick C1 protein reveals insights into the function of the C-terminal luminal domain in cholesterol transport

(2017) Xiaochun Li et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Trifunctional lipid probes for comprehensive studies of single lipid species in living cells

(2017) Doris Höglinger et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Lysosomal cholesterol activates mTORC1 via an SLC38A9–Niemann-Pick C1 signaling complex

(2017) Brian M. Castellano et al.   SCIENCE

Structural basis of dual Ca2+/pH regulation of the endolysosomal TRPML1 channel

(2017) Minghui Li et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

A novel approach to analyze lysosomal dysfunctions through subcellular proteomics and lipidomics: the case of NPC1 deficiency

(2017) Arun Kumar Tharkeshwar et al.   Scientific Reports

High lumenal chloride in the lysosome is critical for lysosome function

(2017) Kasturi Chakraborty et al.   eLife

Autophagy, lipophagy and lysosomal lipid storage disorders

(2016) Carl Ward et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR AND CELL BIOLOGY OF LIPIDS

LDL–cholesterol transport to the endoplasmic reticulum

(2016) Simon G. Pfisterer et al.   CURRENT OPINION IN LIPIDOLOGY

Structural basis for gating the high-conductance Ca2+-activated K+ channel

(2016) Richard K. Hite et al.   NATURE

Cryo-EM structure of the open high-conductance Ca2+-activated K+ channel

(2016) Xiao Tao et al.   NATURE

Clues to the mechanism of cholesterol transfer from the structure of NPC1 middle lumenal domain bound to NPC2

(2016) Xiaochun Li et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

The endoplasmic reticulum, not the pH gradient, drives calcium refilling of lysosomes

(2016) Abigail G Garrity et al.   eLife

A mirror code for protein-cholesterol interactions in the two leaflets of biological membranes

(2016) Jacques Fantini et al.   Scientific Reports

BK channel agonist represents a potential therapeutic approach for lysosomal storage diseases

(2016) Xi Zoë Zhong et al.   Scientific Reports

Autophagy in the light of sphingolipid metabolism

(2015) Eva Bang Harvald et al.   APOPTOSIS

BK Channels Alleviate Lysosomal Storage Diseases by Providing Positive Feedback Regulation of Lysosomal Ca2+ Release

(2015) Qi Cao et al.   DEVELOPMENTAL CELL

Calcium release through P2X4 activates calmodulin to promote endolysosomal membrane fusion

(2015) Qi Cao et al.   JOURNAL OF CELL BIOLOGY

AtomicChargeCalculator: interactive web-based calculation of atomic charges in large biomolecular complexes and drug-like molecules

(2015) Crina-Maria Ionescu et al.   Journal of Cheminformatics

A Voltage-Gated Calcium Channel Regulates Lysosomal Fusion with Endosomes and Autophagosomes and Is Required for Neuronal Homeostasis

(2015) Xuejun Tian et al.   PLOS BIOLOGY

Intracellular sphingosine releases calcium from lysosomes

(2015) Doris Höglinger et al.   eLife

Identification of novel sphingolipid-binding motifs in mammalian membrane proteins

(2014) Patrik Björkholm et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOMEMBRANES

Assessing the local structural quality of transmembrane protein models using statistical potentials (QMEANBrane)

(2014) Gabriel Studer et al.   BIOINFORMATICS

Cooperative Binding of Annexin A2 to Cholesterol- and Phosphatidylinositol-4,5-Bisphosphate-Containing Bilayers

(2014) Patrick Drücker et al.   BIOPHYSICAL JOURNAL

Cholesterol stabilizes fluid phosphoinositide domains

(2014) Zhiping Jiang et al.   CHEMISTRY AND PHYSICS OF LIPIDS

Lysosomal Two-pore Channel Subtype 2 (TPC2) Regulates Skeletal Muscle Autophagic Signaling

(2014) Pei-Hui Lin et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

β-Glucosidase 2 (GBA2) Activity and Imino Sugar Pharmacology

(2013) Christina M. Ridley et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Autophagosomal Syntaxin17-dependent lysosomal degradation maintains neuronal function inDrosophila

(2013) Szabolcs Takáts et al.   JOURNAL OF CELL BIOLOGY

How cholesterol interacts with membrane proteins: an exploration of cholesterol-binding sites including CRAC, CARC, and tilted domains

(2013) Jacques Fantini et al.   Frontiers in Physiology

Phosphatidylinositol 4,5-Biphosphate (PIP2) Lipids Regulate the Phosphorylation of Syntaxin N-Terminus by Modulating Both Its Position and Local Structure

(2012) George Khelashvili et al.   BIOCHEMISTRY

How Slow Is the Transbilayer Diffusion (Flip-Flop) of Cholesterol?

(2012) Theodore L. Steck et al.   BIOPHYSICAL JOURNAL

The Hairpin-type Tail-Anchored SNARE Syntaxin 17 Targets to Autophagosomes for Fusion with Endosomes/Lysosomes

(2012) Eisuke Itakura et al.   CELL

TPC Proteins Are Phosphoinositide- Activated Sodium-Selective Ion Channels in Endosomes and Lysosomes

(2012) Xiang Wang et al.   CELL

δ-Tocopherol Reduces Lipid Accumulation in Niemann-Pick Type C1 and Wolman Cholesterol Storage Disorders

(2012) Miao Xu et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Multiple Cholesterol Recognition/Interaction Amino Acid Consensus (CRAC) Motifs in Cytosolic C Tail of Slo1 Subunit Determine Cholesterol Sensitivity of Ca2+- and Voltage-gated K+(BK) Channels

(2012) Aditya K. Singh et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Molecular recognition of a single sphingolipid species by a protein’s transmembrane domain

(2012) F.-Xabier Contreras et al.   NATURE

Phosphoinositide isoforms determine compartment-specific ion channel activity

(2012) X. Zhang et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Lipid storage disorders block lysosomal trafficking by inhibiting a TRP channel and lysosomal calcium release

(2012) Dongbiao Shen et al.   Nature Communications

Annexin A6 is recruited into lipid rafts of Niemann–Pick type C disease fibroblasts in a Ca2+-dependent manner

(2011) Magdalena M. Domon et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

Molecular Mechanism of Cholesterol- and Polyphosphoinositide-Mediated Syntaxin Clustering

(2011) David H. Murray et al.   BIOCHEMISTRY

Fast, scalable generation of high-quality protein multiple sequence alignments using Clustal Omega

(2011) F. Sievers et al.   Molecular Systems Biology

OPM database and PPM web server: resources for positioning of proteins in membranes

(2011) Mikhail A. Lomize et al.   NUCLEIC ACIDS RESEARCH

Cholesterol Influences Voltage-Gated Calcium Channels and BK-Type Potassium Channels in Auditory Hair Cells

(2011) Erin K. Purcell et al.   PLoS One

Cholesterol Tuning of BK Ethanol Response Is Enantioselective, and Is a Function of Accompanying Lipids

(2011) Chunbo Yuan et al.   PLoS One

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

Disclosure of cholesterol recognition motifs in transmembrane domains of the human nicotinic acetylcholine receptor

(2011) Carlos J. Baier et al.   Scientific Reports

Toward the estimation of the absolute quality of individual protein structure models

(2010) Pascal Benkert et al.   BIOINFORMATICS

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

Purified TPC Isoforms Form NAADP Receptors with Distinct Roles for Ca2+ Signaling and Endolysosomal Trafficking

(2010) Margarida Ruas et al.   CURRENT BIOLOGY

Lysosomal fusion and SNARE function are impaired by cholesterol accumulation in lysosomal storage disorders

(2010) Alessandro Fraldi et al.   EMBO JOURNAL

Specificity of cholesterol and analogs to modulate BK channels points to direct sterol–channel protein interactions

(2010) Anna N. Bukiya et al.   JOURNAL OF GENERAL PHYSIOLOGY

Cholesterol Orientation and Tilt Modulus in DMPC Bilayers

(2010) George Khelashvili et al.   JOURNAL OF PHYSICAL CHEMISTRY B

Combinational Soluble N-Ethylmaleimide-sensitive Factor Attachment Protein Receptor Proteins VAMP8 and Vti1b Mediate Fusion of Antimicrobial and Canonical Autophagosomes with Lysosomes

(2010) Nobumichi Furuta et al.   MOLECULAR BIOLOGY OF THE CELL

PI(3,5)P2 controls membrane trafficking by direct activation of mucolipin Ca2+ release channels in the endolysosome

(2010) Xian-ping Dong et al.   Nature Communications

Clustering of Syntaxin-1A in Model Membranes Is Modulated by Phosphatidylinositol 4,5-Bisphosphate and Cholesterol

(2009) David H. Murray et al.   BIOCHEMISTRY

Jalview Version 2--a multiple sequence alignment editor and analysis workbench

(2009) A. M. Waterhouse et al.   BIOINFORMATICS

pH Dependence of Sphingosine Aggregation

(2009) Hirotaka Sasaki et al.   BIOPHYSICAL JOURNAL

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

(2009) Hyock Joo Kwon et al.   CELL

AutoDock4 and AutoDockTools4: Automated docking with selective receptor flexibility

(2009) Garrett M. Morris et al.   JOURNAL OF COMPUTATIONAL CHEMISTRY

Helical extension of the neuronal SNARE complex into the membrane

(2009) Alexander Stein et al.   NATURE

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

Development of a Rab9 Transgenic Mouse and Its Ability to Increase the Lifespan of a Murine Model of Niemann-Pick Type C Disease

(2008) Tatiana Kaptzan et al.   AMERICAN JOURNAL OF PATHOLOGY

Autophagic dysfunction in mucolipidosis type IV patients

(2008) Silvia Vergarajauregui et al.   HUMAN MOLECULAR GENETICS

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

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

Regulation of the V-ATPase along the Endocytic Pathway Occurs through Reversible Subunit Association and Membrane Localization

(2008) Céline Lafourcade et al.   PLoS One

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

A Specific Cholesterol Binding Site Is Established by the 2.8 Å Structure of the Human β2-Adrenergic Receptor

(2008) Michael A. Hanson et al.   STRUCTURE