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1 tubule fission in lysosome function, similar lysosomal abnormalities were seen in cellular models lac
3 P(E693Q) (Dutch) mutation have intraneuronal lysosomal accumulation of APP carboxylterminal fragments
4 or NPC2 with decreased functions leading to lysosomal accumulation of cholesterol and sphingolipids.
6 e studied the effect of lalistat, a specific lysosomal acid lipase (LAL/Lipa) inhibitor on LD degrada
8 ex vivo activities of debranching enzyme and lysosomal acid maltase, two major hepatic alpha-glucosid
11 sely affecting v-ATPase function dysregulate lysosomal acidification in other LSDs and common neurode
12 wever, lysosomal-delivered MPO also disrupts lysosomal acidification in RPE cells, which coincides wi
14 zed cell was shown to be degraded due to the lysosomal activation in both cells whereas the disintegr
17 E transcription factors-master regulators of lysosomal and melanosomal biogenesis and autophagy-contr
18 TMEM175 plays a direct and critical role in lysosomal and mitochondrial function and PD pathogenesis
19 biochemical studies have implicated impaired lysosomal and mitochondrial function in the pathogenesis
20 Ks) are well characterized for their role in lysosomal and mitochondrial trafficking in cells, especi
21 including specific branches of the endosomal-lysosomal and ubiquitin-proteasome systems, in maintaini
23 lation in the lumen of enlarged perinuclear, lysosomal-associated membrane protein 1 (LAMP1)-positive
24 With respect to one of the target genes, lysosomal beta A mannosidase (MANBA), we observed that g
27 teins occurs by the concurrent activation of lysosomal biogenesis and up-regulation of macroautophagy
28 increased concomitantly with the increase of lysosomal biogenesis induced by lysosome alkalizers or s
29 nted the increase in cellular energy levels, lysosomal biogenesis, and endocytic uptake, suggesting t
30 aster transcriptional regulator of autophagy-lysosomal biogenesis, we can reverse the autophagy dysfu
31 gs suggest that TRPML1 may function as a key lysosomal Ca(2+) channel controlling both lysosome bioge
33 tivation are suppressed by up-regulating the lysosomal Ca(2+) release channel transient receptor pote
37 in several proteins linked to autophagy and lysosomal catabolism reflecting vesicular transport obst
40 PorB increases the level of OVA in the endo-/lysosomal cellular compartment of BMDCs, increases antig
42 ed, fluorescent chloride reporter to measure lysosomal chloride in Caenorhabditis elegans as well as
45 echanisms deliver cytosolic materials to the lysosomal compartment for degradation through autophagy.
46 ce cleaved, the drug escapes the acidic endo-lysosomal compartment into the cytosol and traffics to i
48 me samples, time-dependent activation of the lysosomal compartment with no changes in LC3 expression
50 the most prevalent nuclease activity in endo-lysosomal compartments and that additional stabilization
52 by FBP, aldolases promote the formation of a lysosomal complex containing at least v-ATPase, ragulato
53 AMP-2) immunolabeling showed higher neuronal lysosomal counts in brain of 12-months-old APP(E693Q) as
54 function as a low-affinity Cu transporter, a lysosomal Cu exporter, or a regulator of Ctr1 activity,
56 ragine endopeptidase (AEP) or legumain, is a lysosomal cysteine protease that cleaves both amyloid pr
60 Given the deeper genetic understanding of lysosomal defects in neurodegeneration, we explore why s
63 mpetent DCVs in BON neuroendocrine cells and lysosomal degradation (crinophagy) of insulin-containing
64 between GLP-1R plasma membrane recycling and lysosomal degradation and, in doing so, determine the ov
65 by a double-membrane autophagic process via lysosomal degradation called mitophagy is critical for m
66 ggest an unanticipated function for parasite lysosomal degradation in chronic infection, and identify
67 in-7 and targeted it for internalization and lysosomal degradation in conjunction with claudin-7.
68 ase C inhibitory peptide or bafilomycin A (a lysosomal degradation inhibitor) each blocked the Ang II
72 amine environment, when macropinocytosis and lysosomal degradation of extracellular proteins are used
76 overcompensation by RhoB because of reduced lysosomal degradation of RhoB in Gamma-aminobutyric acid
78 tween mutant and wild-type APP, enhanced the lysosomal degradation of the mutant APP, and inhibited g
79 ated factor (PCAF)-dependent acetylation and lysosomal degradation of the pyruvate kinase-M2 isoform
80 uronate-2-sulfatase, a crucial enzyme in the lysosomal degradation pathway of dermatan sulfate and he
81 NA sensor cyclic GMP-AMP synthase (cGAS) for lysosomal degradation to avoid the detection of mitochon
82 intron of genes that regulate autophagosome/lysosomal degradation, mitochondrial trafficking, and am
83 linked genes play a direct role in autophagy/lysosomal degradation, one of the most important pathway
84 idoglycans restored phagocytic uptake in the lysosomal degradation-defective mutants via a pathway re
96 ul enzyme from the extracellular space, with lysosomal-delivered MPO exhibiting a half-life of 10 h.
97 ow that PGRN facilitates neuronal uptake and lysosomal delivery of prosaposin (PSAP), the precursor o
101 gene therapy) or applicable to more than one lysosomal disorder (haemopoietic stem cell transplantati
103 ase 2 clinical trials) might be for specific lysosomal disorders (enzyme replacement therapy via intr
105 and mammalian cell culture models of diverse lysosomal disorders, where previously only lysosomal pH
107 of TFEB in JAK2-deficient podocytes reversed lysosomal dysfunction and restored albumin permselectivi
108 In Drosophila and mammalian macrophages, lysosomal dysfunction due to loss of the endolysosomal C
110 Amyloid precursor protein and endosomal-lysosomal dysfunction in Alzheimer's disease: inseparabl
112 id metabolism associated with late endosomal/lysosomal dysfunction may play a role in the pathogenesi
113 LP-1 receptor agonist exendin-4 reversed the lysosomal dysfunction, relieving the impairment in autop
116 Inhibition of mTOR strongly reduced the lysosomal efflux of most essential amino acids, converti
117 n lysosomal pH, which leads to impairment of lysosomal enzyme activity and disruption of autophagic p
118 ulti-system disorder caused by deficiency of lysosomal enzyme alpha-L-iduronidase, and patients treat
120 link between mutations in GBA1, encoding the lysosomal enzyme glucocerebrosidase, and the synucleinop
122 (WT) decreases lysotracker signal, secreted lysosomal enzymes and SNAP23-mediated lysosome exocytosi
124 106b(-/-) mice, we show that, while multiple lysosomal enzymes are increased in Grn(-/-) brain at bot
125 ysosome and impedes the activity of specific lysosomal enzymes indicating a broader role for chloride
126 ndrome, MPS IIIA-D, results from deficits in lysosomal enzymes that specifically degrade heparan sulf
128 noparticle (NP) enabling excellent endosomal/lysosomal escape and efficient siRNA decomplexation insi
129 usly showed that modulation of autophagy and lysosomal exocytosis by overexpression of the transcript
131 endocytosis of protein-bound Cbl followed by lysosomal export of free Cbl to the cytosol and further
132 a secretory lysosomal protein that regulates lysosomal function and biogenesis by controlling the aci
133 tinophagy, suggesting that alpha-syn impairs lysosomal function by disrupting the trafficking of lyso
135 e suggests that PGRN is essential for proper lysosomal function, but the precise mechanisms involved
138 tive payloads, strategies aimed at restoring lysosomal functionality might overcome resistance to ADC
140 ing role for lysosomes and shed new light on lysosomal functions during intracellular Ca(2+) homeosta
142 naling plays a crucial role in inhibition of lysosomal fusion and autolysosomal destruction of ehrlic
144 early endosome-like vacuoles and circumvents lysosomal fusion through an unknown mechanism, thereby a
145 ptor signaling component Dishevelled induced lysosomal fusion with ehrlichial inclusions correspondin
146 demonstrate that neuronal activity triggers lysosomal fusion with the plasma membrane in dendrites.
148 with Wnt5a demonstrated increased endosomal/lysosomal fusions with parasite-containing vacuoles (par
149 r insufficiency of PGRN led to the increased lysosomal gene expression and protein levels, while PGRN
151 d led to downregulation in the expression of lysosomal genes and decreased activity of the lysosomal
152 ng enzymes, glucosylceramide synthase (GCS), lysosomal glucosylceramidase (GBA), and the cytosolic re
154 an important mechanism of how p97 maintains lysosomal homeostasis, and implicate the pathway as a mo
155 demonstrate that hENT3 is indispensable for lysosomal homeostasis, and that mutations in hENT3 can r
157 Here, we studied CTSB regulation by another lysosomal hydrolase, cathepsin D (CTSD), using mice with
158 some fusion (PLF) results in the delivery of lysosomal hydrolases into phagosomes and in digestion of
164 internalized proteopathic seeds, and suggest lysosomal integrity as a significant rate-determining st
166 Transmembrane protein 175 (TMEM175), the lysosomal K(+) channel, is centered under a major genome
167 of alpha-SYN in the astrocytes affects their lysosomal machinery and induces mitochondrial damage.
169 tor NBR1, the autophagy protein LC3, and the lysosomal marker LAMP1 to Mtb-associated structures and
170 lichial vacuoles did not colocalize with the lysosomal marker LAMP2, and lysosomes were redistributed
173 ibility loci and reinforce the importance of lysosomal mechanisms in Parkinson's disease pathogenesis
174 LP-1 mediates beta-cell survival via the key lysosomal-mediated process of autophagy is unknown.
175 permeation pathway for glutamine across the lysosomal membrane and it is required for growth of canc
177 findings indicate that while autophagosomal-lysosomal membrane fusion is sensitive to inhibition of
180 mutant form of Bax is sufficient to increase lysosomal membrane permeability and restore autophagic c
182 as well as induction of ER stress, leads to lysosomal membrane permeabilization (LMP), a sustained a
183 Accumulation of defective lysosomes leads to lysosomal membrane permeabilization and release of cathe
184 ginine requires SLC38A9, a poorly understood lysosomal membrane protein with homology to amino acid t
188 Thus, through SLC38A9, arginine serves as a lysosomal messenger that couples mTORC1 activation to th
189 r potential mucolipin 1 (TRPML1) but not the lysosomal Na(+) release channel two-pore channel 2 (TPC2
190 lian TPC channels have been shown to be endo/lysosomal Na(+)-selective or Ca(2+)-release channels.
193 key role for proteolysis within the parasite lysosomal organelle (the vacuolar compartment or VAC) in
194 ing RagA GTPase recruitment of mTORC1 to the lysosomal outer surface, enabling activation of mTOR by
196 ry pathways and identified an unconventional lysosomal pathway as an important mechanism for mHtt sec
197 endoplasmic reticulum (ER) to the endosomal/lysosomal pathway by transiently binding DP(84Gly) via a
202 es involved in the endocytotic and endosomal/lysosomal pathways, and disruption of miR-153 expression
205 e lysosomal disorders, where previously only lysosomal pH dysregulation has been described, massive r
207 as also shown to cause additional changes in lysosomal pH, which leads to impairment of lysosomal enz
210 for pff uptake in primary neurons, implicate lysosomal processing as the major fate of internalized p
214 al axis involves suppression of proapoptotic lysosomal protein cathepsin D by promotion of the ER-ass
215 and YopD colocalized with the late endosomal/lysosomal protein LAMP1 and that the frequency of YopD a
217 m106b deletion from Grn(-/-) mice normalizes lysosomal protein levels and rescues FTLD-related behavi
221 termates, and western blots showed increased lysosomal proteins including LAMP-2, cathepsin D and LC3
222 n host cells deficient for late endosomal or lysosomal proteins revealed that the Niemann-Pick type C
226 C38A9 is necessary for leucine generated via lysosomal proteolysis to exit lysosomes and activate mTO
227 rotease deficiency in lysosomes and impaired lysosomal proteolysis, as evidenced by aberrant accumula
232 We also found mislocalization of the CMA lysosomal receptor LAMP2A and impaired substrate translo
233 omal biogenesis and autophagy-control mTORC1 lysosomal recruitment and activity by directly regulatin
234 conjugation, a monodisperse drug loading, a lysosomal release functionality and monomethyl auristati
236 the pathway lead to harmful accumulation of lysosomal sphingolipid species, which are associated wit
237 demonstrated defects in autophagic flux and lysosomal staining in human samples of type 2 diabetes.
242 een previously associated with the endosomal/lysosomal storage diseases Niemann-Pick and neuronal cer
245 Niemann-Pick disease, type C1 (NPC1) is a lysosomal storage disorder characterised by progressive
246 ype C1 (NPC1) disease is a neurodegenerative lysosomal storage disorder due to mutations in the NPC1
247 significant burden of rare, likely damaging lysosomal storage disorder gene variants in association
248 used to interrogate variant burden among 54 lysosomal storage disorder genes, leveraging whole exome
249 encodes TRPML1, cause the neurodegenerative lysosomal storage disorder mucolipidosis type IV, and a
250 expression and lead to the neurodegenerative lysosomal storage disorder neuronal ceroid lipofuscinosi
251 rare autosomal recessive, neurodegenerative lysosomal storage disorder, which presents with a range
253 l acidification contributes to virtually all lysosomal storage disorders (LSDs) and to common neurode
256 as been implicated in human diseases such as lysosomal storage disorders, neurodegenerative diseases
261 coincides with nuclear translocation of the lysosomal stress-sensing transcription factor EB and, ev
263 embrane protein TMEM55B recruits JIP4 to the lysosomal surface, inducing dynein-dependent transport o
266 and recruits GATOR1, but not GATOR2, to the lysosomal surface; and is necessary for the interaction
270 We show here that OGD causes endocytosis, lysosomal targeting and consequent degradation of GluA2-
271 3 complex was impaired, leading to increased lysosomal targeting and reduced surface levels on CD4(+)
272 Importantly, we found that late endosomal/lysosomal targeting and secretion of mHtt could be inhib
273 ngs identify new mechanisms whereby impaired lysosomal targeting can impact the activity and recyclin
276 an unanticipated role of Cln1 in regulating lysosomal targeting of V0a1 and suggest that varying fac
278 subcellular redistribution of CTSD from the lysosomal to the zymogen-containing subcellular compartm
279 we demonstrate that dense core vesicles and lysosomal trafficking dynamics are affected in fibroblas
282 isorder caused by biallelic mutations in the lysosomal trafficking regulator gene (LYST), resulting i
283 , using Lyst-mutant beige mice, we show that lysosomal trafficking regulator Lyst links endolysosomal
284 nal water channel aquaporin-2 (AQP2) and the lysosomal trafficking regulator-interacting protein LIP5
291 complex (BORC), previously shown to regulate lysosomal transport, is required to recruit and activate
292 7 (SNAT7), encoded by the SLC38A7 gene, as a lysosomal transporter highly selective for glutamine and
293 t at pH 7.4, hENT3 is an acidic pH-activated lysosomal transporter partially localized to mitochondri
294 This assay was used to screen candidate lysosomal transporters, leading to the identification of
297 ate endosome/lysosome and interacts with the lysosomal v-ATPase to negatively regulate mTORC1 activat
299 king, the cellular machinery associated with lysosomal vesicles that regulates their docking and secr
300 protein Vamp-7 is associated with Lamp-1(+) lysosomal vesicles, which are recruited and docked at th
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