コーパス検索結果 (1語後でソート)
通し番号をクリックするとPubMedの該当ページを表示します
5 ions in the GAA gene, resulting in deficient lysosomal acid-alpha-glucosidase activity in patients, a
7 based dynamic assembly system that achieves lysosomal acidic microenvironment specifically inducing
9 of mTORC1-ATF4 hyperactivation and impaired lysosomal acidification in beta-cells lacking Furin, cau
13 oded ratiometric pHlys biosensor, pHLARE (pH Lysosomal Activity REporter), which localizes predominan
14 acropinocytosis of CPPs results in increased lysosomal activity, NLRP3 inflammasome activation, and I
15 this by regulating mitochondrial fission and lysosomal activity, suppressing glucose uptake, and main
19 -EM structures of Rags in complex with their lysosomal anchor complex Ragulator and the cytoplasmic t
20 ctivates TFEB, which increases expression of lysosomal and autophagosomal genes, and that TFEB activa
21 dysregulation of genes within the endosomal-lysosomal and autophagy pathways, cytoskeletal elements,
25 in the galactosidase beta1 (GLB1) gene cause lysosomal beta-gal deficiency, resulting in accumulation
26 n the galactosidase beta 1 (GLB1) gene cause lysosomal beta-galactosidase (beta-Gal) deficiency and c
27 es raise questions about the identity of the lysosomal binding partner of the C9orf72 complex and the
28 tion factor EB (TFEB), a master regulator of lysosomal biogenesis and autophagy(4,5), is phosphorylat
29 n factors TFEB and TFE3-master regulators of lysosomal biogenesis and autophagy-control ER-phagy by i
30 (TFEB)-a master transcriptional regulator of lysosomal biogenesis and autophagy-is activated during t
33 B (TFEB) functions as a master controller of lysosomal biogenesis and function during lysosomal stres
35 ng pathways; in turn, the nucleus fine-tunes lysosomal biogenesis and functions through transcription
37 w that the light-responsive miR-211 controls lysosomal biogenesis at the beginning of light-dark tran
38 he autophagosomal pathway and the control of lysosomal biogenesis by TFEB, thus ensuring coordinated
39 dysfunction induced by gene inactivations of lysosomal biogenesis or acidification factors causes vit
42 tion factor EB (TFEB), a master regulator of lysosomal biogenesis, plays an essential role in the lys
43 a network of genes involved in autophagy and lysosomal biogenesis, were examined in the context of C.
47 , phagocytosis is driven by these local endo-lysosomal Ca(2+) nanodomains rather than global cytoplas
48 imaging with genetics, we here identify the lysosomal Ca2+ channel Trpml as an essential player in t
49 omes, where lipidated LC3 interacts with the lysosomal calcium channel TRPML1, facilitating calcium e
51 g mitochondrial calcium dynamics through the lysosomal calcium efflux channel, transient receptor pot
53 inhibitors of parasite cathepsins L and host lysosomal cathepsin L, S and K cysteine proteases (inhib
54 now demonstrate that an interaction with the lysosomal cationic amino acid transporter PQLC2 mediates
56 at plays a crucial role in the metabolism of lysosomal ceramides, important members of the sphingolip
63 ally distinct catabolic pathways that ensure lysosomal clearance of a model organelle, the endoplasmi
64 has been argued to be critical for efficient lysosomal clearance; however, its context to autophagic
65 to investigate the impact of PARK2 KO on the lysosomal compartment and found a clear link between par
66 H84T is internalized into the late endosomal/lysosomal compartment and inhibits virus-endosome fusion
68 show that DSTYK is located in late endosomal/lysosomal compartments and is involved in the lysosome b
69 uantitative image analysis, we show that the lysosomal content and activity are globally elevated in
70 d electron-lucent lysosomes and an increased lysosomal content, which was exacerbated by mitochondria
71 torage disorder caused by dysfunction of the lysosomal cotransporter cystinosin, leads to cystine acc
74 placement, were detected in model systems of lysosomal damage inflicted by proteopathic tau and durin
75 enesis and autophagy-is activated during the lysosomal damage response, and its activation is depende
77 levels, pathogens, energy availability, and lysosomal damage, with the goal of restoring cellular ho
83 dition, elevating miR-195 rescues AD-related lysosomal defects in inducible pluripotent stem cells (i
84 rgeting of LAMTOR1 recapitulated most of the lysosomal defects of targeting NMT1, including defective
85 ocesses that encompasses insulin resistance, lysosomal defects, decreased survival factors, increased
86 MHC-I molecules are selectively targeted for lysosomal degradation by an autophagy-dependent mechanis
87 in lysosome deacidification, inactivation of lysosomal degradation enzymes, and disruption of antigen
90 pathway cannot go to completion (e.g., when lysosomal degradation is impaired) may instead exacerbat
93 here that autophagy, a pathway allowing the lysosomal degradation of intracellular components, plays
94 ng it release through exosomes and promoting lysosomal degradation of misfolded prion proteins in can
98 of the perturbed AP-1 sorting, m154 promotes lysosomal degradation of several proteins involved in T
100 cally promotes ubiquitination and subsequent lysosomal degradation of the LDLR and thus controls cell
105 directing secreted and membrane proteins for lysosomal degradation, with broad implications for bioch
114 which cytoplasmic contents are targeted for lysosomal digestion, has homeostatic functions including
115 aracterized, autosomal recessively inherited lysosomal disease caused by mutations in lysosomal traff
116 BAQ13 ONNs are highly effective in inducing lysosomal disruption, lysosomal dysfunction and autophag
117 contained macrophages with hyperacetylated, lysosomal disulfide-HMGB1 that increased postreperfusion
119 effective in inducing lysosomal disruption, lysosomal dysfunction and autophagy blockade and exhibit
122 growing evidence indicates that the role of lysosomal dysfunction in human diseases goes beyond rare
126 is sufficient to recapitulate inflammation, lysosomal dysfunction, and hyperproliferation in a cell-
127 including the oxidative stress, DNA damage, lysosomal dysfunction, inflammatory cascade, apoptosis,
133 ar acetylome, with chloroquine, to alter the lysosomal environment to favor stability of the traffick
134 tions in the GBA gene, which encodes for the lysosomal enzyme beta-glucocerebrosidase (GCase), result
136 Mutations in the GBA gene, which encodes the lysosomal enzyme glucocerebrosidase (GCase), are the mos
138 age disorder caused by the deficiency of any lysosomal enzyme required for the breakdown of glycosami
139 ficiency results in inefficient ER export of lysosomal enzymes and diminished levels of the enzymes a
141 mes of the lysosomal system), which recruits lysosomal enzymes at the ER to promote their Golgi trans
144 nd supporting the concept that TFEB-mediated lysosomal exocytosis promotes cellular clearance, we sho
145 lymphocytes, and electron microscopy showed lysosomal (fingerprint) inclusions in all 8 patients.
147 ect of advanced aging, genetic disruption of lysosomal function accelerated the accumulation of stora
148 hosphate multikinase, promotes autophagy and lysosomal function and biogenesis in a TFEB-dependent ma
149 tablishes a membrane potential essential for lysosomal function and its dysregulation is associated w
150 this network and associated receptors modify lysosomal function and subsequently impact both SNCA deg
151 at common variability at genes implicated in lysosomal function exerts the largest effect on GBA asso
152 that pharmacological interventions targeting lysosomal function in general, and those particularly ca
153 wever, mechanisms linking nutrient-dependent lysosomal function to insulin secretion and more general
161 strated new analytical tools to characterize lysosomal functions and microenvironment in human neuron
164 y, the degradation of cytoplasmic content by lysosomal fusion, is an evolutionary conserved process p
166 PFFs, but not monomeric alpha-syn, inhibited lysosomal GCase activity in these cells and induced the
168 ought to better understand the regulation of lysosomal genes whose expression does not appear to be c
172 Galactosidase (beta-gal), one of the typical lysosomal glycosidases, is reported to be a vital biomar
173 oton exchanger CLC-7 plays critical roles in lysosomal homeostasis and bone regeneration and its muta
175 PreC layer III projection neurons contain lysosomal hydrolase cathepsin D (CatD), a marker of neur
176 Mutations in GBA1, the gene encoding the lysosomal hydrolase glucocerebrosidase (GCase), are a ri
177 ue to the secretion and subsequent uptake of lysosomal hydrolases by adjacent cells, often referred t
179 al inhibitors MG132 and ALLN rather than the lysosomal inhibitors leupeptin and pepstatin A, suggesti
181 of both known and new lysosomal membrane and lysosomal-interacting proteins in human iPSC-derived neu
182 Here we show that ion selectivity of the lysosomal ion channel TPC2, which is hotly debated (Calc
183 r findings uncover a broadened PSEN1 role in lysosomal ion homeostasis and novel pH modulation of lys
186 odeling, molecular dynamics simulations, and lysosomal localization experiments were performed on TME
187 fied ATXN3 as a Ub-Rheb deubiquitinase whose lysosomal localization is blocked by active Rag heterodi
188 to decreased Raptor acetylation and reduced lysosomal localization of mTOR, resulting in impaired ac
189 WDR41 and the DENN domain of SMCR8 drive the lysosomal localization of the complex in conditions of a
190 onductance of the transmembrane protein, and lysosomal localization was not affected by these variant
195 hich localized to active puncta that promote lysosomal-mediated stimulator of interferon genes (STING
196 complementary coverage of both known and new lysosomal membrane and lysosomal-interacting proteins in
197 ted to also interact with the late endosomal/lysosomal membrane glycoprotein CLN3 (ceroid lipofuscino
201 is a K(+)-selective ion channel expressed in lysosomal membranes, where it establishes a membrane pot
205 lockade of its degradation instigates robust lysosomal mTORC1 localization and its activation without
209 beta-Coronavirus-induced exploitation of lysosomal organelles for egress provides insights into t
210 f several GTPases essential to the endosomal-lysosomal pathway and is implicated in protein secretion
213 Loss of GCase activity impairs the autophagy lysosomal pathway resulting in increased alpha-synuclein
214 UB1 plays a role in regulating the autophagy-lysosomal pathway when the ubiquitin proteasome system i
215 a process of degradation that occurs via the lysosomal pathway, has an essential role in multiple asp
219 ecrosis from impaired energy homeostasis and lysosomal permeabilization and inflammation through the
220 IL-1beta/TNF-induced necrosis resulted from lysosomal permeabilization and release of cathepsins B a
222 are important for autophagosome turnover and lysosomal pH regulation and are associated with the earl
223 HeLa cells localizes to lysosomes and raises lysosomal pH, suggesting that in melanocytes SLC45A2 exp
225 wn to exhibit homology to LCAT and mammalian lysosomal PLA(2) , and to contain a conserved and functi
227 titer genotypes, while membrane trafficking, lysosomal processes, and mechanistic target of rapamycin
228 in is involved in endocytosis, secretion and lysosomal processes, but its functions under physiologic
230 esource, we detected aberrant trafficking of lysosomal proteases to the extracellular space and the n
233 insight into the relationship between these lysosomal proteins, GCase, and SNCA, and reveal novel th
234 fibroblasts revealed extensive missorting of lysosomal proteins, including sphingolipid catabolism en
237 and pharmacologic mTORC1 inhibition restores lysosomal proteolysis without correcting cholesterol sto
238 m PSEN1 familial AD patients, which restores lysosomal proteolysis, calcium homeostasis, and normal a
242 lacking SL-1, MtbDeltapks2, shows attenuated lysosomal rewiring compared with the WT Mtb in both in v
243 of USP10 or G3BP1 family proteins increased lysosomal ribosomal degradation and perturbed ribosomal
245 show that the cytoplasmic tail of the human lysosomal solute carrier family 38 member 9 (SLC38A9) de
248 ed macrophages and suggest that this altered lysosomal state protects host cell integrity and contrib
249 reas Clcn6(-/-) mice have only mild neuronal lysosomal storage abnormalities, the affected individual
250 ns of the forebrain demonstrated progressive lysosomal storage and microglial activation despite a la
251 and its mutation can lead to osteopetrosis, lysosomal storage disease and neurological disorders.
252 s an autosomal recessive, neurodegenerative, lysosomal storage disease caused by mutations in CLN3, w
253 NEU1 is deficient in the neurodegenerative lysosomal storage disease sialidosis, and its targeting
254 B (Sanfilippo syndrome B; OMIM 252920), is a lysosomal storage disease with progressive neurological
259 gagement in two animal models of neuropathic lysosomal storage diseases (LSDs), Gaucher's and Krabbe'
263 ilippo syndrome B) is an autosomal recessive lysosomal storage disorder caused by the deficiency of a
265 Batten disease (CLN3 disease) is a pediatric lysosomal storage disorder that presents with progressiv
267 This model could be extrapolated to other lysosomal storage disorders in which immune response hin
269 goes beyond rare inherited diseases, such as lysosomal storage disorders, to include common neurodege
271 throughout the CNS including accumulation of lysosomal storage material and glial activation, and has
272 etected in the cerebellum, where progressive lysosomal storage, astrocytosis and microglial activatio
274 urthermore, we find that cholesterol-induced lysosomal stress feed-forward activates TFEB via promoti
275 of lysosomal biogenesis and function during lysosomal stress, controlling most but, importantly, not
278 ts reveal that global alteration of the host lysosomal system is a defining feature of Mtb-infected m
280 RESS: ER-to-Golgi relaying of enzymes of the lysosomal system), which recruits lysosomal enzymes at t
285 deficient ubiquitin-proteasome and autophagy-lysosomal systems), neuroinflammation and oxidative stre
286 e DNA repair nuclease MRE11A and inefficient lysosomal tethering of AMPK due to deficiency of N-myris
287 teractions and becomes associated with known lysosomal trafficking and proteolytic proteins like SQST
288 demonstrate that beta-coronaviruses utilize lysosomal trafficking for egress rather than the biosynt
292 sm introducing a premature stop codon in the lysosomal trafficking regulator gene (LYST) that shorten
293 We recently revealed that the late endo-lysosomal transporter ATP13A2 pumps polyamines like sper
296 nase (AMPK) inhibitor dorsomorphin decreased lysosomal V-ATPase activity and also blocked any increas
297 blocks the starvation-dependent increase in lysosomal V-ATPase activity without altering basal activ
298 red for the starvation-dependent increase in lysosomal V-ATPase activity, indicating that H89 and dor
299 sponsible for the sorting of proteins to the lysosomal vacuole is Rsp5, a member of the Nedd4 family