Mitochondrial Dysfunction and Neurodegeneration in Lysosomal Storage Disorders

Parkin is recruited selectively to impaired mitochondria and promotes their autophagy

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TFEB Links Autophagy to Lysosomal Biogenesis

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Fission and selective fusion govern mitochondrial segregation and elimination by autophagy

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PINK1 Is Selectively Stabilized on Impaired Mitochondria to Activate Parkin

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A Gene Network Regulating Lysosomal Biogenesis and Function

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Prevalence of lysosomal storage disorders

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Multicenter Analysis of Glucocerebrosidase Mutations in Parkinson's Disease

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A lysosome‐to‐nucleus signalling mechanism senses and regulates the lysosome via mTOR and TFEB

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Signals from the lysosome: a control centre for cellular clearance and energy metabolism

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A facile soft-template synthesis of mesoporous polymeric and carbonaceous nanospheres

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A century of trends in adult human height

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A forty-kilodalton protein of the inner membrane is the mitochondrial calcium uniporter

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Lysosomal calcium signalling regulates autophagy through calcineurin and TFEB

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MTORC1 functions as a transcriptional regulator of autophagy by preventing nuclear transport of TFEB

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Selective Mitochondrial Autophagy, or Mitophagy, as a Targeted Defense Against Oxidative Stress, Mitochondrial Dysfunction, and Aging

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Gaucher disease glucocerebrosidase and α-synuclein form a bidirectional pathogenic loop in synucleinopathies

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Metabolic control of mitochondrial biogenesis through the PGC-1 family regulatory network

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Lysosomal acidification mechanisms

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The axonal transport of mitochondria

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The Coactivator PGC-1 Cooperates with Peroxisome Proliferator-Activated Receptor α in Transcriptional Control of Nuclear Genes Encoding Mitochondrial Fatty Acid Oxidation Enzymes

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Trehalose, a Novel mTOR-independent Autophagy Enhancer, Accelerates the Clearance of Mutant Huntingtin and α-Synuclein

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TFEB controls cellular lipid metabolism through a starvation-induced autoregulatory loop

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Sorting of lysosomal proteins

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Regulation of glucose metabolism from a liver-centric perspective

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

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α-Synuclein impairs macroautophagy: implications for Parkinson’s disease

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AMPK activators: mechanisms of action and physiological activities

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Optineurin is an autophagy receptor for damaged mitochondria in parkin-mediated mitophagy that is disrupted by an ALS-linked mutation

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Genome-Wide Association Study of Retinopathy in Individuals without Diabetes

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A Vesicular Transport Pathway Shuttles Cargo from Mitochondria to Lysosomes

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Lysosomal storage disorders: The cellular impact of lysosomal dysfunction

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Direct Membrane Association Drives Mitochondrial Fission by the Parkinson Disease-associated Protein α-Synuclein*♦

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The Mitochondrial Permeability Transition Pore: Channel Formation by F-ATP Synthase, Integration in Signal Transduction, and Role in Pathophysiology

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MCOLN1 is a ROS sensor in lysosomes that regulates autophagy

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Correction: Corrigendum: Differential developmental requirement and peripheral regulation for dermal Vγ4 and Vγ6T17 cells in health and inflammation

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A block of autophagy in lysosomal storage disorders

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Reduced glucocerebrosidase is associated with increased α-synuclein in sporadic Parkinson’s disease

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Different Responses of Astrocytes and Neurons to Nitric Oxide: The Role of Glycolytically Generated ATP in Astrocyte Protection

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Lipofuscin: Formation, Distribution, and Metabolic Consequences

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Lysosomal storage diseases: from pathophysiology to therapy

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The Cl-/H+ antiporter ClC-7 is the primary chloride permeation pathway in lysosomes

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Mitophagy programs: mechanisms and physiological implications of mitochondrial targeting by autophagy

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Identification and characterization of a proton pump on lysosomes by fluorescein-isothiocyanate-dextran fluorescence.

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mTOR Regulates Lysosomal ATP-Sensitive Two-Pore Na+ Channels to Adapt to Metabolic State

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Mitochondrial ATP synthase subunit c storage in the ceroid‐lipofuscinoses (Batten disease)

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Presenilin 1 Maintains Lysosomal Ca2+ Homeostasis via TRPML1 by Regulating vATPase-Mediated Lysosome Acidification

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Mitochondria and Quality Control Defects in a Mouse Model of Gaucher Disease—Links to Parkinson’s Disease

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Mitochondrial Respiration Controls Lysosomal Function during Inflammatory T Cell Responses

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Reducing C-Terminal-Truncated Alpha-Synuclein by Immunotherapy Attenuates Neurodegeneration and Propagation in Parkinson's Disease-Like Models

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Acid β‐glucosidase mutants linked to gaucher disease, parkinson disease, and lewy body dementia alter α‐synuclein processing

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Chromerid genomes reveal the evolutionary path from photosynthetic algae to obligate intracellular parasites

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Impairing the bioenergetic status and the biogenesis of mitochondria triggers mitophagy in yeast

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GM1-Ganglioside Accumulation at the Mitochondria-Associated ER Membranes Links ER Stress to Ca2+-Dependent Mitochondrial Apoptosis

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Mitochondrial calcium uniporter Mcu controls excitotoxicity and is transcriptionally repressed by neuroprotective nuclear calcium signals

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Autophagy Is Disrupted in a Knock-in Mouse Model of Juvenile Neuronal Ceroid Lipofuscinosis

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Up-regulation of lysosomal TRPML1 channels is essential for lysosomal adaptation to nutrient starvation

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Loss of Mitochondrial Function Impairs Lysosomes

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[17] Imaging mitochondrial function in intact cells

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TRP-ML1 Regulates Lysosomal pH and Acidic Lysosomal Lipid Hydrolytic Activity

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TMEM175 Is an Organelle K+ Channel Regulating Lysosomal Function

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MCUR1 Is a Scaffold Factor for the MCU Complex Function and Promotes Mitochondrial Bioenergetics

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Optical Reporters for the Conformation of α-Synuclein Reveal a Specific Interaction with Mitochondria

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Altered Cholesterol Metabolism in Niemann-Pick Type C1 Mouse Brains Affects Mitochondrial Function

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The birth prevalence of lysosomal storage disorders in the Czech Republic: comparison with data in different populations

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

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The m -AAA Protease Associated with Neurodegeneration Limits MCU Activity in Mitochondria

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Ca2+ is a key factor in α-synuclein-induced neurotoxicity

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Elevation of Intracellular Glucosylceramide Levels Results in an Increase in Endoplasmic Reticulum Density and in Functional Calcium Stores in Cultured Neurons

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Glucocerebrosidase depletion enhances cell-to-cell transmission of α-synuclein

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The regulation of neuronal mitochondrial metabolism by calcium

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HMGB1 is involved in autophagy inhibition caused by SNCA/α-synuclein overexpression

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Mitochondrial trafficking and the provision of energy and calcium buffering at excitatory synapses

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NRF2 Orchestrates the Metabolic Shift during Induced Pluripotent Stem Cell Reprogramming

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Enhanced calcium release in the acute neuronopathic form of Gaucher disease

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Neuroinflammation, mitochondrial defects and neurodegeneration in mucopolysaccharidosis III type C mouse model

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Mitochondrial Aberrations in Mucolipidosis Type IV

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Inhibition of Calcium Uptake via the Sarco/Endoplasmic Reticulum Ca2+-ATPase in a Mouse Model of Sandhoff Disease and Prevention by Treatment with N-Butyldeoxynojirimycin

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Intracellular sphingosine releases calcium from lysosomes

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Follow-up study of subunit c of mitochondrial ATP synthase (SCMAS) in Batten disease and in unrelated lysosomal disorders

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Membrane trafficking and mitochondrial abnormalities precede subunit c deposition in a cerebellar cell model of juvenile neuronal ceroid lipofuscinosis

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Mitochondria in Developmental and Adult Neurogenesis

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Lysosome-Mitochondrial Crosstalk in Cellular Stress and Disease

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A zebrafish screen reveals Renin-angiotensin system inhibitors as neuroprotective via mitochondrial restoration in dopamine neurons

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Targeted Metabolomic Analysis of a Mucopolysaccharidosis IIIB Mouse Model Reveals an Imbalance of Branched-Chain Amino Acid and Fatty Acid Metabolism

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Altered Sphingolipids Metabolism Damaged Mitochondrial Functions: Lessons Learned From Gaucher and Fabry Diseases

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Caspase-8 Induces Lysosome-Associated Cell Death in Cancer Cells

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Targetable Pathways for Alleviating Mitochondrial Dysfunction in Neurodegeneration of Metabolic and Non-Metabolic Diseases

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Key Mechanisms in Lysosome Stability, Degradation and Repair

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An Alzheimer's Disease-Linked Loss-of-Function CLN5 Variant Impairs Cathepsin D Maturation, Consistent with a Retromer Trafficking Defect

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Clinical Trials for Gene Therapy in Lysosomal Diseases With CNS Involvement

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Aminochrome Induces Irreversible Mitochondrial Dysfunction by Inducing Autophagy Dysfunction in Parkinson's Disease

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Nicotinamide Riboside and Metformin Ameliorate Mitophagy Defect in Induced Pluripotent Stem Cell-Derived Astrocytes With POLG Mutations

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Linking Lysosomal Enzyme Targeting Genes and Energy Metabolism with Altered Gray Matter Volume in Children with Persistent Stuttering

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Brain RNA-Seq Profiling of the Mucopolysaccharidosis Type II Mouse Model

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Lysosomal Storage Disorders Shed Light on Lysosomal Dysfunction in Parkinson’s Disease

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Perspectives on Organelle Interaction, Protein Dysregulation, and Cancer Disease

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Fabry Disease: Cardiac Implications and Molecular Mechanisms

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Fundamentals of CNS energy metabolism and alterations in lysosomal storage diseases

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Finding pathogenic commonalities between Niemann-Pick type C and other lysosomal storage disorders: Opportunities for shared therapeutic interventions

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Rescue of Advanced Pompe Disease in Mice with Hepatic Expression of Secretable Acid α-Glucosidase

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Potential Treatment of Lysosomal Storage Disease through Modulation of the Mitochondrial—Lysosomal Axis

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When friendship turns sour: Effective communication between mitochondria and intracellular organelles in parkinson’s disease

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Secondary Mitochondrial Dysfunction as a Cause of Neurodegenerative Dysfunction in Lysosomal Storage Diseases and an Overview of Potential Therapies

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Fabry Disease: Insights into Pathophysiology and Novel Therapeutic Strategies

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Fabry Disease Beyond Storage: The Role of Inflammation in Disease Progression

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Association Between Gray Matter Volume Variations and Energy Utilization in the Brain: Implications for Developmental Stuttering

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Bu-Yin-Qian-Zheng Formula Ameliorates MPP+-Induced Mitochondrial Dysfunction in Parkinson’s Disease via Parkin

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Organelle dysfunction and its contribution to metabolic impairments in aging and age-related diseases

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Mitochondrial complex I density is associated with IQ and cognition in cognitively healthy adults: an in vivo [18F]BCPP-EF PET study

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sPLA2-IIA modifies progranulin deficiency phenotypes in mouse models

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β-Mannosidosis in German Shepherd Dogs

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Learning Nanoscale Motion Patterns of Vesicles in Living Cells

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Evolving therapies in neuronopathic LSDs: opportunities and challenges

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Decoding the muscle transcriptome of patients with late-onset Pompe disease reveals markers of disease progression

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4-Phenylbutyric acid mitigates ER stress-induced neurodegeneration in the spinal cords of a GM2 gangliosidosis mouse model

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Methylmercury induces lysosomal membrane permeabilization through JNK-activated Bax lysosomal translocation in neuronal cells

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Mitochondrial respiratory chain regulates HBV clearance through dual modulation of lysosomal acidification

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Organellomics: AI-driven deep organellar phenotyping reveals novel ALS mechanisms in human neuronsPreprint

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