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1         Defective cystine transport leads to intralysosomal accumulation and crystallization of cysti
2 xohydrolase that, when deficient, results in intralysosomal accumulation of heparan sulfate and the c
3                          SPNS1 loss leads to intralysosomal accumulation of lysophosphatidylcholine (
4 ciencies in normal lysosomal function and by intralysosomal accumulation of undegraded substrates.
5  glycosaminoglycan catabolism, causing their intralysosomal accumulation.
6 se and neuraminidase and is required for the intralysosomal activity and stability of these two glyco
7  of inherited disorders characterized by the intralysosomal buildup of partially degraded metabolites
8                                          The intralysosomal Ca(2+) content and receptor-mediated Ca(2
9 ns may have resulted in stimulus-independent intralysosomal Ca(2+) release, hence the surface express
10 omal neuraminidase is the key enzyme for the intralysosomal catabolism of sialylated glycoconjugates
11 ysosomes together with alkalinization of the intralysosomal compartment would result in an overall de
12               These results suggest that the intralysosomal compartments can accumulate extensive amo
13 t) or transgenic protein overexpression, the intralysosomal content of alpha-synuclein was also signi
14  CTNS lead to defective cystinosin function, intralysosomal cystine accumulation and the development
15                                              Intralysosomal cystine crystal accumulation, due to muta
16                                          The intralysosomal digestion of endocytosed [3H]RNase A was
17 n in cytosolic Fe(2+) levels, an increase in intralysosomal Fe(2+) levels and an accumulation of lipo
18  cells to phagocytose collagen fibrils whose intralysosomal hydrolysis was prevented in the presence
19 e, by monitoring the levels of cytosolic and intralysosomal iron and by directly patch-clamping the l
20  levels and sensitized TM cells to H(2)O(2), intralysosomal iron chelation completely protected the c
21 iven by reactive oxygen species (ROS), is an intralysosomal, non-degradable, auto-fluorescent macromo
22 ed to lysosomal nucleoside buildup, elevated intralysosomal pH, and altered macrophage function.
23           This is due to an elevation of the intralysosomal pH, since ionophores and proton pump inhi
24 P-dependent proton pump V-ATPase ensures low intralysosomal pH, which is essential for lysosomal hydr
25                                   NPC2 is an intralysosomal protein that binds cholesterol in vitro.
26                              NPC2 is a small intralysosomal protein that has been characterized bioch
27 hese results indicate that the generation of intralysosomal ROS induces lysosomal membrane permeabili
28 somes and proteolytically processed, but its intralysosomal stability is intermediate between that of
29 eukodystrophy (MLD) characterized by massive intralysosomal storage of the acidic glycosphingolipid s
30 al neurodegenerative disease presenting with intralysosomal storage, enhances clearance of proteolipi
31 cysteine transporter MFSD12 was required for intralysosomal transport of NAC and rescue of LLP.
32 y, degradation takes place at the surface of intralysosomal vesicles of late endosomes and lysosomes
33 ion of GM2AP is to bind and extract GM2 from intralysosomal vesicles, forming a soluble protein-lipid
34 hosphate (BMP), a major lipid constituent of intralysosomal vesicles, stimulates lipid-degrading enzy