AnatomyOrganismsDiseasesChemicals and DrugsAnalytical, Diagnostic and Therapeutic Techniques and EquipmentPhenomena and ProcessesDisciplines and OccupationsInformation ScienceHealth Care
LysosomesProteasome Endopeptidase ComplexEndosomesProteolysisLysosome-Associated Membrane GlycoproteinsUbiquitinEndocytosisAutophagyPhagosomesBiodegradation, EnvironmentalUbiquitinationAcid PhosphataseCell LineCathepsin DProtein TransportMolecular Sequence DataUbiquitin-Protein LigasesCysteine EndopeptidasesAmino Acid SequenceLysosomal-Associated Membrane Protein 2Cells, CulturedCathepsinsKineticsMicroscopy, ElectronVacuolesUbiquitinsMutationMultienzyme ComplexesChloroquineEndoplasmic ReticulumBiological TransportEndoplasmic Reticulum-Associated DegradationProtein BindingHydrogen-Ion ConcentrationHydrolysisProtein Processing, Post-TranslationalLiverSubcellular FractionsHeLa CellsGolgi ApparatusFibroblastsPeptide HydrolasesCell MembraneCell FractionationProteinsModels, Biologicalbeta-N-AcetylhexosaminidasesOrganellesMembrane ProteinsCathepsin Brab GTP-Binding ProteinsChediak-Higashi SyndromeMicroscopy, FluorescenceSignal TransductionHydrolasesReceptor, IGF Type 2Cell CompartmentationCysteine Proteinase InhibitorsGlucuronidaseTime FactorsProtein Structure, TertiaryEndopeptidasesIntracellular MembranesCarrier ProteinsCytoplasmLysosomal Storage DiseasesAmmonium ChlorideRecombinant Fusion ProteinsMacrophagesProtease InhibitorsProteasome InhibitorsRNA, MessengerMannosephosphatesOrganoidsMucolipidosesPhosphorylationEndosomal Sorting Complexes Required for TransportCell Line, TumorMultivesicular BodiesCricetinaeHEK293 CellsRNA StabilityAsialoglycoproteinsBlotting, WesternBase SequenceMicroscopy, ConfocalAndrostenesVesicular Transport ProteinsCathepsin LCytosolF-Box ProteinsRNA InterferenceSubstrate SpecificityAcetylglucosaminidaseElectrophoresis, Polyacrylamide GelDown-RegulationHexosaminidasesProtein StabilityRNA, Small InterferingRecombinant Proteins
Lysosomal degradationFuseAutophagosomeCytosolicEndocytosisMembrane-bound compartmentsEndosomesPathwayCytoplasmicSequestrationCellularProteins and organellesOrganellesProteasomesAccumulationSubstratesEndoplasmicProteinReceptor-mediatedEndogenousPathogensDysfunctionCompartmentsAcidicMutationsFusesBuildupBiochemicalCellsEnzymesCytoplasmIntracellularCytosolDigestionMechanismsFragmentsDisordersCellBasalContentsAffectsFormInductionSpecificBody
Lysosomal degradation3
  • Autophagy is a conserved catabolic process that eliminates dysfunctional cytosolic biomolecules through vacuole-mediated sequestration and lysosomal degradation. (hindawi.com)
  • Autophagy is a cellular catabolic process that eliminates damaged cell organelles, unfolded proteins, and various intracellular pathogens through lysosomal degradation. (hindawi.com)
  • Our projects investigating lysosomal stability will contribute to finding strategies how to increase lysosomal degradation and clearance and might thus be useful in both neurodegenerative diseases and lysosomal storage disorders. (liu.se)
Fuse8
Autophagosome7
  • Once the autophagosome forms, it fuses with the lysosome and creates a structure called the autolysosome. (hindawi.com)
  • During autophagosome maturation, cargo recruitment takes place through the recruitment of ATGs and LC3, and mature autophagosome formation occurs. (hindawi.com)
  • Ultimately, the autophagosome fuses with a lysosome, facilitated by Rab7 and LAMP proteins. (hindawi.com)
  • The autophagophore membrane then elongates and encloses the molecules to be degraded forming an autophagosome, which occurs in two separate conjugation reactions catalyzed by autophagy-related proteins (ATGs). (hindawi.com)
  • Autophagy is an intracellular catabolic pathway in which cellular constituents are engulfed by autophagosomes and degraded upon autophagosome fusion with lysosomes. (nature.com)
  • Autophagy/macroautophagy is a lysosome-dependent catabolic process characterized by increased formation of double-membrane autophagosomes for the sequestration of cytoplasmic components and subsequent degradation after autophagosome fusion with lysosomes 1 , 2 . (nature.com)
  • EV-D68 uses virally-encoded proteases to cleave an autophagosome fusion SNARE protein, SNAP29, blocking delivery of autophagosome contents, including nascent viruses, to the lysosome. (umaryland.edu)
Cytosolic4
  • Autophagy is a regulated process of cytosolic degradation in eukaryotic cells which maintains cellular homeostasis by degrading damaged organelles, protein aggregates, microbes and other xenobiotics in the cytoplasm. (umaryland.edu)
  • The autophagic process is characterized by the formation of double-membraned autophagosomes around cytosolic cargo, which then undergo a series of fusion steps with endosomes and lysosomes to degrade the vesicle's contents. (umaryland.edu)
  • Autophagy allows the degradation of cytosolic endogenous and exogenous material in the lysosome. (lih.lu)
  • Autophagy, an evolutionarily conserved process, is characterized by massive degradation of cytosolic contents. (spandidos-publications.com)
Endocytosis4
  • Lysosomes are acidic organelles that are critically involved in a number of physiological processes including macromolecule degradation, endocytosis, autophagy, exocytosis and cholesterol homeostasis. (liu.se)
  • As nouns the difference between endosome and phagosome is that endosome is (biology) an endocytic vacuole through which molecules internalized during endocytosis pass en route to lysosomes while phagosome is a membrane-bound vacuole within a cell containing foreign material captured by phagocytosis. (mybubbaandme.com)
  • Specific endocytosis and degradation of naked DNA in the endocardial cells of cod (Gadus morhua L. (silverchair.com)
  • In the absence of neurons, the major myelin membrane protein, the proteolipid protein (PLP), is internalized and stored in late endosomes/lysosomes (LEs/Ls) by a cholesterol-dependent and clathrin-independent endocytosis pathway that requires actin and the RhoA guanosine triphosphatase. (rupress.org)
Membrane-bound compartments2
  • Lysosomes are membrane-bound compartments that degrade macromolecules and clear damaged organelles to enable cellular adaptation to various metabolic states. (stanford.edu)
  • In normal physiology, the lysosomes are membrane bound compartments which break down nutrients such as fats, carbohydrates, and proteins. (kireportscommunity.org)
Endosomes1
  • Cell surface Gb3 is constitutively endocytosed to late endosomes/lysosomes (LEL) (blue arrows). (eucor-uni.org)
Pathway6
  • The autophagy pathway is targeted by many pathogens, either to protect themselves from degradation or to utilize components to benefit replication. (umaryland.edu)
  • Using this strategy, we uncovered a basal, housekeeping mitophagy pathway that involves piecemeal degradation of mitochondrial proteins in a LC3C- and p62-dependent manner and contributes to mitochondrial homeostasis maintenance when cells rely on oxidative phosphorylation. (lih.lu)
  • Macro)Autophagy is a catabolic pathway that delivers excess, aggregated, or damaged proteins and organelles to lysosomes for degradation. (figshare.com)
  • The N-terminal arginylation of proteins is characterized as an inducer of ubiquitination and proteasomal degradation by the N-end rule pathway. (mit.edu)
  • Because the main pathway for glycogen degradation is not deficient in glycogen-storage disease type II disease, energy production is not impaired, and hypoglycemia does not occur. (medscape.com)
  • Next, they review the growing evidence that the lysosome-mediated degradation pathway autophagy plays a central role in synaptic maintenance, and how the disruption in autophagy may contribute to early cognitive changes in HD. (press-news.org)
Cytoplasmic4
  • First, phagophore formation requires cytoplasmic vesicle nucleation, which occurs when the ATG1/ULK1 complex, which is comprised of ULK1, FIP200, ATG13, and ATG101, interacts with the Class III PI3K complex. (hindawi.com)
  • It involves the retention of cytoplasmic material into autophagosomes for degradation by lysosomes. (epigentek.com)
  • Autophagy is a major intracellular degradative process that delivers cytoplasmic materials to the lysosome for degradation. (nature.com)
  • Half a century ago, Christian de Duve coined the term "autophagy" (literally, "self-eating" in Greek) to describe a process where the cell digests its cytoplasmic materials within lysosomes 1 . (nature.com)
Sequestration1
  • Starting with sequestration, transport to lysosomes, degradation and utilization of degradation products, each sequential step is also vital to the autophagy process. (bodyecology.com)
Cellular11
  • After the degradation of damaged proteins and lipids, amino acids and fatty acids are released into the cytoplasm and recycled for new biosynthesis of cellular components or energy production [ 4 ]. (hindawi.com)
  • The cellular role of acid alpha-glucosidase is to convert glycogen into glucose within the lysosomes. (medscape.com)
  • Acid alpha-glucosidase (also known as acid maltase) is a lysosomal hydrolase that is required for the degradation of a small percentage (1-3%) of cellular glycogen. (medscape.com)
  • Excessive glycogen storage within lysosomes interrupts normal functioning of other organelles and leads to cellular injury. (medscape.com)
  • The lysosome contains over 50 different hydrolases responsible for its degradative function and a single membrane surrounds it, which is heavily glycosylated to protect other cellular compartments from its hydrolytic enzymes. (liu.se)
  • Lysosomes are key components of many cellular processes, which make them attractive therapeutic targets. (liu.se)
  • By developing novel tools and harnessing the power of metabolomics, proteomics and functional genomics, our lab will define 1) how the lysosome communicates with other cellular compartments to fulfill the metabolic demands of the cell under various metabolic states, 2) and how its dysfunction leads to rare and common human diseases. (stanford.edu)
  • Lysosomes are involved in cellular digestion. (wikipedia.org)
  • The AAA-ATPase (ATPases associated with diverse cellular activities) valosin-containing protein (VCP), is essential for many cellular pathways including but not limited to endoplasmic reticulum-associated degradation (ERAD), DNA damage responses, and cell cycle regulation. (portlandpress.com)
  • CFAB specifically decreases cellular melanin by most likely inducing rapid degradation of tyrosinase in B16 cells. (elsevierpure.com)
  • In the work presented in this dissertation, we found that increasing LDLR levels enhanced the cellular uptake and degradation of amyloid beta by primary astrocytes, and increased amyloid beta transport to lysosomes. (wustl.edu)
Proteins and organelles1
  • Autophagy is generally considered as a cell survival/protection mechanism because it removes toxic or obsolete proteins and organelles and recycles the degradation products for use as sources for energy and metabolites in anabolic pathways 3 . (nature.com)
Organelles2
Proteasomes2
  • The degradation of misfolded/unfolded proteins is an essential element of proteostasis and occurs mainly in lysosomes or proteasomes. (mit.edu)
  • Interestingly, unlike other reagents that promote degradation of tyrosinase in proteasomes or lysosomes, neither proteasomal nor lysosomal inhibitors can halt CFAB-induced tyrosinase degradation. (elsevierpure.com)
Accumulation4
  • However, the deficiency of acid alpha-glucosidase activity does result in the accumulation of structurally normal glycogen in lysosomes and in the cytoplasm of affected individuals. (medscape.com)
  • Lysosomal storage disorders represent a class of inborn pathologies characterized by the accumulation of material in lysosomes. (liu.se)
  • Although autophagy occurs in the cytoplasm, its inhibition leads to accumulation of DNA damage and genomic instability. (figshare.com)
  • These mutations cause a defect in cystinosin, leading to a cystine accumulation and subsequent crystallization that occurs during protein degradation transport. (kireportscommunity.org)
Substrates1
  • VCP interacts with adaptor proteins to identify ubiquitylated substrates for degradation by the proteasome. (portlandpress.com)
Endoplasmic2
Protein4
  • While it is not known how the loss of this protein causes the signs and symptoms of CLN6 disease, it is likely that the protein's quick degradation contributes to the childhood onset of CLN6 disease. (medlineplus.gov)
  • Cystinosis is caused by mutations in the CTNS gene which codes for the protein, cystinosin, - a hydrogen ion-driven transporter that removes cystine from lysosomes. (kireportscommunity.org)
  • The encoded protein functions as a cell surface hyaluronidase that cleaves extracellular high molecular weight hyaluronan into intermediate size fragments before internalization and degradation in the lysosome. (modellab.cn)
  • The authors conclude that there are pathologic and imaging data in individuals with mutations in the Huntingtin protein (mHtt), as well as evidence from animal models with HD, that suggest that synapse dysfunction may occur early in HD, prior to cell death. (press-news.org)
Receptor-mediated1
  • Phagocytosis is a term coined from the Greek to describe the receptor-mediated engulfment and degradation of dead cells and microbes. (frontiersin.org)
Endogenous1
  • Today, the most promising therapy for lysosomal storage disorders including alpha-Mannosidosis is Enzyme Replacement Therapy (ERT) where the respective enzyme lacking in the patient is produced by recombinant approaches and then introduced into the blood stream, from where it is internalized by the cells and reaches the lysosomes replacing the missing endogenous enzyme. (europa.eu)
Pathogens1
  • The lysosomes contain hydrolytic enzymes and reactive oxygen species (ROS) which kill and digest the pathogens. (mybubbaandme.com)
Dysfunction2
  • Due to the essential role of lysosomes in autophagy, lysosomal dysfunction impairs this process, thereby contributing to disease. (liu.se)
  • Lysosomal function is critical for organismal homeostasis-mutations in genes encoding lysosomal proteins cause severe human disorders known as lysosomal storage diseases, and lysosome dysfunction is implicated in age-associated diseases including cancer, neurodegeneration and metabolic syndrome. (stanford.edu)
Compartments1
  • These results suggest that CFAB-induced tyrosinase decrease occurs in post-Golgi compartments but not in proteasomal or lysosomal compartments. (elsevierpure.com)
Acidic1
  • One possibility is that sphingolipids could lessen the activity of vacuolar ATPase, the proton pump in lysosomes that maintains their acidic internal environment. (alzforum.org)
Mutations2
  • However, it is unclear how mutations in the CLN6 gene are involved in the buildup of substances in lysosomes in CLN6 disease. (medlineplus.gov)
  • Naturally occurring mutations in this gene are associated with autosomal recessive non-syndromic hearing loss. (modellab.cn)
Fuses1
Buildup1
Biochemical2
  • To address these questions, our lab uses a multidisciplinary approach to study the biochemical functions of the lysosome in vitro and in vivo. (stanford.edu)
  • We study the role of the lysosome in metabolic adaptation using subcellular omics approaches, functional genomics and innovative biochemical tools. (stanford.edu)
Cells6
  • The lysosome is a compartment that functions as the place where degradation of the cells takes place during autophagy. (bodyecology.com)
  • It occurs within the cells of everything from the brain to the liver, muscles and skin. (bodyecology.com)
  • Rapidly dividing cells, such as cancer cells, are highly dependent on effective lysosomal function and dramatic changes in lysosomal volume, composition, and subcellular localization occur during transformation and cancer progression. (liu.se)
  • Fabry cells lack alpha-Gal A and thus inhibit Gb3 degradation and re-utilization of the lipid components. (eucor-uni.org)
  • These accumulations occur in more cells throughout the body in children with CLN6 disease than in affected adults. (medlineplus.gov)
  • Since virus release from insect cells occurs by budding, these results suggest that both proteins are FCCP site involved in budding, and thus also suggests a specific role for NS3a. (adenosine-receptor.com)
Enzymes2
Cytoplasm1
Intracellular1
  • In general, autophagy degrades long-lived damaged intracellular proteins, in contrast to the ubiquitin-proteasome system, which controls the degradation of short-lived proteins [ 2 ]. (hindawi.com)
Cytosol1
  • The fully functional autolysosome then degrades the autolysosomal cargo and releases the degradation products into the cytosol for recycling. (hindawi.com)
Digestion1
  • There, Gb3 is degraded by the enzyme alpha-Gal A. This digestion triggers the total degradation of Gb3 to various components. (eucor-uni.org)
Mechanisms1
  • Common mechanisms of recognition, engulfment, and degradation of the different types of cargo are assumed, but very little is known about the shared and specific molecules involved in the phagocytosis of each target by microglia. (frontiersin.org)
Fragments1
  • In radiolabeling experiments, Tamboli and colleagues showed that this overabundance of APP-CTFs occurred because the fragments were less efficiently degraded. (alzforum.org)
Disorders1
  • Lysosomes have a central role in lysosomal storage disorders and increasing evidence indicates that lysosomes are involved also in more widespread diseases, such as cancer, and neurodegenerative diseases. (liu.se)
Cell2
  • Most of these proteins mature in the Golgi apparatus before going to their final destination which may be to lysosomes , peroxisomes , or outside of the cell. (wikipedia.org)
  • This preprint looks at how SARS-CoV-2 infection modulates arginylation, a modification that tags proteins for degradation, and finds a specific arginylation signature in some cell types. (mit.edu)
Basal1
  • Autophagy occurs during normal development at the basal level, as well as under stress conditions. (nature.com)
Contents1
  • Phagosomes then interact with lysosomes to form phagolysosomes, resulting in the degradation of the phagosomal contents. (mybubbaandme.com)
Affects1
  • It affects about 1 in 100,000-200,000 people in the general population,occurs in all ethnic groups, and affects males and females equally. (kireportscommunity.org)
Form2
  • In the classic infantile form of Pompe disease, clinically significant glycogen storage occurs in cardiac muscle. (medscape.com)
  • In the infantile form, clinically significant storage occurs in the heart, resulting in progressive cardiomegaly with left ventricular (LV) thickening that eventually leads to outflow tract obstruction. (medscape.com)
Induction1
  • Our data show that relocalization occurs for SNAP47 during autophagy induction, and is required for normal virus replication. (umaryland.edu)
Specific2
  • Depending on the involvement of specific receptor proteins, autophagy occurs in a selective or nonselective manner. (lih.lu)
  • Whatever we take for the beginning of old age - be it, say, the appearance of some specific disease or just the end of reproduction - we will quickly find out that none of these signs occur overnight. (vechnayamolodost.ru)
Body1