Evidence
Biochim Biophys Acta Mol Basis Dis. 2023 May 18:166756. doi: 10.1016/j.bbadis.2023.166756. Online ahead of print.
ABSTRACT
• Neuronal Ceroido Lipofuscinoses (NCL) are inherited, neurodegenerative disorders associated with lysosomal storage. • Impaired autophagy plays a pathogenetic role in several NCL forms, including CLN3 disease, but study on human brains lacks. • In post-mortem brain samples of a CLN3 patient the LC3-I to LC3-II shift was consistent with activated autophagy. However, the autophagic process seemed to be ineffective due to the presence of lysosomal storage markers. • After fractionation with buffers of increasing detergent-denaturing strength, a peculiar solubility pattern of LC3-II was observed in CLN3 patient’s samples, suggesting a different lipid composition of the membranes where LC3-II is stacked.
PMID:37209872 | DOI:10.1016/j.bbadis.2023.166756
Estimated reading time: 2 minute(s)
Latest: Psychiatryai.com #RAISR4D Evidence
Cool Evidence: Engaging Young People and Students in Real-World Evidence
Real-Time Evidence Search [Psychiatry]
AI Research
Enhanced expression of the autophagosomal marker LC3-II in detergent-resistant protein lysates from a CLN3 patient’s post-mortem brain
🌐 90 Days
AI Virtual Reality Related Evidence Matrix
- Lysosomal Channels as New Molecular Targets in the Pharmacological Therapy of Neurodegenerative Diseases via Autophagy Regulation
- Copper enhances aggregational toxicity of mutant huntingtin in a Drosophila model of Huntington's Disease
- Mutations in CLCN6 as a Novel Genetic Cause of Neuronal Ceroid Lipofuscinosis
- Reduction of spermine synthase enhances autophagy to suppress Tau accumulation
- miR-519a-3p, found to regulate cellular prion protein during Alzheimer's disease pathogenesis, as a biomarker of asymptomatic stages
- Alterations of human CSF and serum-based mitophagy biomarkers in the continuum of Alzheimer disease
- β-Hydroxybutyrate and melatonin suppress maladaptive UPR, excessive autophagy and pyroptosis in Aβ 1-42 and LPS-Induced SH-SY5Y cells
- Varicella zoster virus-induced autophagy in human neuronal and hematopoietic cells exerts antiviral activity
- Optogenetic manipulation of lysosomal physiology and autophagy-dependent clearance of amyloid beta
- APOE4 dysregulates autophagy in cultured cells
- APOE4 dysregulates autophagy in cultured cells
- ROS-mediated lysosomal membrane permeabilization and autophagy inhibition regulate bleomycin-induced cellular senescence
- Transcriptomic sex differences in postmortem brain samples from patients with psychiatric disorders
- Transcriptomic sex differences in postmortem brain samples from patients with psychiatric disorders
- TFEB activator 1 enhances autophagic degradation of oligomeric amyloid-β in microglia
- AAV-mediated gene therapy for Sialidosis
- Autophagy preferentially degrades non-fibrillar polyQ aggregates
- Cyanidin-3-O-glucoside alleviates ethanol-induced liver injury by promoting mitophagy in a Gao-binge mouse model of alcohol-associated liver disease
- Unraveling the link between Neuropathy Target Esterase NTE/SWS, lysosomal storage diseases, inflammation, abnormal fatty acid metabolism, and leaky brain barrier
- Tcf4 dysfunction alters dorsal and ventral cortical neurogenesis in Pitt-Hopkins syndrome mouse model showing sexual dimorphism
- A genetically-encoded fluorescence-based reporter to spatiotemporally investigate mannose-6-phosphate pathway
- An initial HOPS-mediated fusion event is critical for autophagosome transport initiation from the axon terminal
- ADNP dysregulates methylation and mitochondrial gene expression in the cerebellum of a Helsmoortel-Van der Aa syndrome autopsy case
- Tau fibrils evade autophagy by excessive p62 coating and TAX1BP1 exclusion
- Exercised-enriched blood plasma rescues hippocampal impairments and cognitive deficits in an Alzheimer's disease model
- The new kids on the block: RNA-binding proteins regulate autophagy in disease
- Ratiometric fluorescence imaging of lysosomal NO in living cells and mice brains with Alzheimer's disease
- Transcriptomic characterization of human lateral septum neurons reveals conserved and divergent marker genes across species
- Inhibition of autophagy prevents cardiac dysfunction at early stages of cardiomyopathy in Bag3-deficient hearts
- The Spectrum of Cerebral Small Vessel Disease: Emerging Pathophysiologic Constructs and Management Strategies
- In situ temperature determination using magnetic resonance spectroscopy thermometry for noninvasive postmortem examinations
- Systemic immune challenge exacerbates neurodegeneration in a model of neurological lysosomal disease
- Systemic immune challenge exacerbates neurodegeneration in a model of neurological lysosomal disease
- Perinatal loss of galactosylceramidase in both oligodendrocytes and microglia is crucial for the pathogenesis of Krabbe disease in mice
- Perinatal loss of galactosylceramidase in both oligodendrocytes and microglia is crucial for the pathogenesis of Krabbe disease in mice
- Glial Markers of Suicidal Behavior in the Human Brain-A Systematic Review of Postmortem Studies
- Disruption of a DNA G-quadruplex causes a gain-of-function SCL45A1 variant relevant to developmental disorders
- Biochemical, Biomarker, and Behavioral Characterization of the GrnR493X Mouse Model of Frontotemporal Dementia
- Mouse serum albumin induces neuronal apoptosis and tauopathies
- TMEM106B C-terminal fragments aggregate and drive neurodegenerative proteinopathy
- Lysosomal dysfunction and overload of nucleosides in thymidine phosphorylase deficiency of MNGIE
- DDHD2 promotes lipid droplet catabolism by acting as a TAG lipase and a cargo receptor for lipophagy
- Performance Metrics, Algorithms, and Applications of Artificial Intelligence in Vascular and Interventional Neurology: A Review of Basic Elements
- Fatty acid-binding protein 5 is a functional biomarker and indicator of ferroptosis in cerebral hypoxia
- ER-to-lysosome Ca2+ refilling followed by K+ efflux-coupled store-operated Ca2+ entry in inflammasome activation and metabolic inflammation
- The necroptosis cell death pathway drives neurodegeneration in Alzheimer's disease
- The necroptosis cell death pathway drives neurodegeneration in Alzheimer's disease
- Old age alters inflammation and autophagy signaling in the brain, leading to exacerbated neurological outcomes after spinal cord injury in male mice
- Development, validation and application of single molecule molecular inversion probe based novel integrated genetic screening method for 29 common lysosomal storage disorders in India
- New perspectives on the role of biological factors in anorexia nervosa: Brain volume reduction or oxidative stress, which came first?
- New perspectives on the role of biological factors in anorexia nervosa: Brain volume reduction or oxidative stress, which came first?
- Antipsychotic Zuclopenthixol Inhibits Melanoma Growth and Brain Metastasis by Inducing Apoptosis and Cell Cycle Arrest
- The overexpression of DSP1 in neurons induces neuronal dysfunction and neurodegeneration phenotypes in Drosophila
- Evaluation of [(18)F]fluoroestradiol and ChRERalpha as a gene expression PET reporter system in rhesus monkey brain
- A current view of mitochondria damage and the diversity of lipopigment inclusions in neuronal ceroid lipofuscinose type 2 from rectal biopsy
- Evaluation of [18F]fluoroestradiol and ChRERα as a gene expression PET reporter system in rhesus monkey brain
- Biallelic variants in LARS1 induce steatosis in developing zebrafish liver via enhanced autophagy
- Biallelic variants in LARS1 induce steatosis in developing zebrafish liver via enhanced autophagy
- Influence of human gut microbiome on the healthy and the neurodegenerative aging
- Dissecting the MUC5AC/ANXA2 signaling axis: implications for brain metastasis in lung adenocarcinoma
- Alteration of gene expression and protein solubility of the PI 5-phosphatase SHIP2 are correlated with Alzheimer's disease pathology progression
- Alteration of gene expression and protein solubility of the PI 5-phosphatase SHIP2 are correlated with Alzheimer's disease pathology progression
- DHCR7 links cholesterol synthesis with neuronal development and axonal integrity
- Pongamol Alleviates Neuroinflammation and Promotes Autophagy in Alzheimer's Disease by Regulating the Akt/mTOR Signaling Pathway
Evidence Blueprint
Enhanced expression of the autophagosomal marker LC3-II in detergent-resistant protein lysates from a CLN3 patient’s post-mortem brain
☊ AI-Driven Related Evidence Nodes
(recent articles with at least 5 words in title)
More Evidence
Enhanced expression of the autophagosomal marker LC3-II in detergent-resistant protein lysates from a CLN3 patient’s post-mortem brain
🌐 365 Days
AI Virtual Reality Related Evidence Matrix
- Exercise ameliorates lipid droplet metabolism disorder by the PLIN2-LIPA axis-mediated lipophagy in mouse model of non-alcoholic fatty liver disease
- Lysosomal Channels as New Molecular Targets in the Pharmacological Therapy of Neurodegenerative Diseases via Autophagy Regulation
- Accumulation of neutral lipids in dystrophic neurites surrounding amyloid plaques in Alzheimer's disease
- Copper enhances aggregational toxicity of mutant huntingtin in a Drosophila model of Huntington's Disease
- Mutations in CLCN6 as a Novel Genetic Cause of Neuronal Ceroid Lipofuscinosis
- Reduction of spermine synthase enhances autophagy to suppress Tau accumulation
- miR-519a-3p, found to regulate cellular prion protein during Alzheimer's disease pathogenesis, as a biomarker of asymptomatic stages
- Autophagy Markers Are Altered in Alzheimer's Disease, Dementia with Lewy Bodies and Frontotemporal Dementia
- Disruption of lysosomal nutrient sensing scaffold hyperactivates mTORC1 contributing to pathogenesis of a fatal neurodegenerative disease
- Autophagy and exosomes; inter-connected maestros in Alzheimer's disease
- Phosphorylation-state dependent intraneuronal sorting of Aβ differentially impairs autophagy and the endo-lysosomal system
- Inhibition of autophagic flux by S-nitrosylation of SQSTM1/p62 promotes neuronal secretion and cell-to-cell transmission of SNCA/α-synuclein in Parkinson disease and Lewy body dementia
- Alterations of human CSF and serum-based mitophagy biomarkers in the continuum of Alzheimer disease
- β-Hydroxybutyrate and melatonin suppress maladaptive UPR, excessive autophagy and pyroptosis in Aβ 1-42 and LPS-Induced SH-SY5Y cells
- Varicella zoster virus-induced autophagy in human neuronal and hematopoietic cells exerts antiviral activity
- Optogenetic manipulation of lysosomal physiology and autophagy-dependent clearance of amyloid beta
- CASM mediates LRRK2 recruitment and activation under lysosomal stress
- Neurologic involvement in cystinosis: Focus on brain lesions and new evidence of four-repeat (4R-) Tau immunoreactivity
- Endoglin and soluble endoglin in liver sinusoidal endothelial dysfunction in vivo
- Therapeutic targeting of HYPDH/PRODH2 with N-propargylglycine offers a Hyperoxaluria treatment opportunity
- The beneficial role of autophagy in multiple sclerosis: yes or no?
- APOE4 dysregulates autophagy in cultured cells
- APOE4 dysregulates autophagy in cultured cells
- Nicotinic acetylcholine receptors in cancer: Limitations and prospects
- Alcohol induces hepatocytes necroptosis through the LC3/RIPK1/RIPK3 pathway
- Dem-Aging: autophagy-related pathologies and the "two faces of dementia"
- Metformin rescues migratory deficits of cells derived from patients with periventricular heterotopia
- White adipose tissue: Distribution, molecular insights of impaired expandability, and its implication in fatty liver disease
- Single nuclei transcriptomics in diabetic mice reveals altered brain hippocampal endothelial cell function, permeability, and behavior
- Impact of non-alcoholic fatty liver disease (NAFLD) and non-alcoholic steatohepatitis (NASH) on the expression and function of hepatobiliary transporters: A comprehensive mechanistic review
- ROS-mediated lysosomal membrane permeabilization and autophagy inhibition regulate bleomycin-induced cellular senescence
- Correction of symptoms of Huntington disease by genistein through FOXO3-mediated autophagy stimulation
- Transcriptomic sex differences in postmortem brain samples from patients with psychiatric disorders
- Transcriptomic sex differences in postmortem brain samples from patients with psychiatric disorders
- TFEB activator 1 enhances autophagic degradation of oligomeric amyloid-β in microglia
- Targeting autophagy to counteract neuroinflammation: a novel antidepressant strategy
- AAV-mediated gene therapy for Sialidosis
- Mechanism of Shenling Baizhu Powder on treatment of alcoholic liver disease by regulating TLR4/NLRP3 pathway
- Autophagy preferentially degrades non-fibrillar polyQ aggregates
- 4R MAPT/tau drives endolysosomal and autophagy dysfunction in frontotemporal dementia
- Role of autophagy in betaine-promoted hepatoprotection against non-alcoholic fatty liver disease in mice
- Polypathologic associations with grey matter atrophy in neurodegenerative disease
- Headache in Brain Tumors
- Epigenetic regulation of autophagy-related genes: Implications for neurodevelopmental disorders
- Cyanidin-3-O-glucoside alleviates ethanol-induced liver injury by promoting mitophagy in a Gao-binge mouse model of alcohol-associated liver disease
- Neuronal Gtf2i deletion alters mitochondrial and autophagic properties
- Dynamics of cellular plasticity in non-alcoholic steatohepatitis (NASH)
- p-tau Ser356 is associated with Alzheimer's disease pathology and is lowered in brain slice cultures using the NUAK inhibitor WZ4003
- Unraveling the link between Neuropathy Target Esterase NTE/SWS, lysosomal storage diseases, inflammation, abnormal fatty acid metabolism, and leaky brain barrier
- A toxic gain-of-function mechanism in C9orf72 ALS impairs the autophagy-lysosome pathway in neurons
- Targeted degradation of SNCA/α-synuclein aggregates in neurodegeneration using the AUTOTAC chemical platform
- Naringin Protects against Non-Alcoholic Fatty Liver Disease by Promoting Autophagic Flux and Lipophagy
- Translocator protein is a marker of activated microglia in rodent models but not human neurodegenerative diseases
- Cathepsin D mediates prenatal caffeine exposure-caused NAFLD susceptibility in male rat offspring by regulating autophagy
- Transcription factor EB: A potential integrated network regulator in metabolic-associated cardiac injury
- Advancements in high-resolution 3D microscopy analysis of endosomal morphology in postmortem Alzheimer's disease brains
- Disease and brain region specific immune response profiles in neurodegenerative diseases with pure and mixed protein pathologies
- Tcf4 dysfunction alters dorsal and ventral cortical neurogenesis in Pitt-Hopkins syndrome mouse model showing sexual dimorphism
- A genetically-encoded fluorescence-based reporter to spatiotemporally investigate mannose-6-phosphate pathway
- An initial HOPS-mediated fusion event is critical for autophagosome transport initiation from the axon terminal
- Hepatic Steatosis Alleviated by a Novel Metformin and Quercetin Combination Activating Autophagy Through the cAMP/AMPK/SIRT1 Pathway
- Muscle diffusion MRI reveals autophagic buildup in a mouse model for Pompe disease
- ADNP dysregulates methylation and mitochondrial gene expression in the cerebellum of a Helsmoortel-Van der Aa syndrome autopsy case
- Drosophila melanogaster models of MPS IIIC (Hgsnat-deficiency) highlight the role of glia in disease presentation