ライフサイエンスコーパス: intralysosomal

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1 xohydrolase that, when deficient, results in intralysosomal accumulation of heparan sulfate and the c

2 ciencies in normal lysosomal function and by intralysosomal accumulation of undegraded substrates.

3 glycosaminoglycan catabolism, causing their intralysosomal accumulation.

4 se and neuraminidase and is required for the intralysosomal activity and stability of these two glyco

5 of inherited disorders characterized by the intralysosomal buildup of partially degraded metabolites

6 The intralysosomal Ca(2+) content and receptor-mediated Ca(2

7 ns may have resulted in stimulus-independent intralysosomal Ca(2+) release, hence the surface express

8 omal neuraminidase is the key enzyme for the intralysosomal catabolism of sialylated glycoconjugates

9 ysosomes together with alkalinization of the intralysosomal compartment would result in an overall de

10 These results suggest that the intralysosomal compartments can accumulate extensive amo

11 t) or transgenic protein overexpression, the intralysosomal content of alpha-synuclein was also signi

12 CTNS lead to defective cystinosin function, intralysosomal cystine accumulation and the development

13 Intralysosomal cystine crystal accumulation, due to muta

14 The intralysosomal digestion of endocytosed [3H]RNase A was

15 n in cytosolic Fe(2+) levels, an increase in intralysosomal Fe(2+) levels and an accumulation of lipo

16 cells to phagocytose collagen fibrils whose intralysosomal hydrolysis was prevented in the presence

17 e, by monitoring the levels of cytosolic and intralysosomal iron and by directly patch-clamping the l

18 levels and sensitized TM cells to H(2)O(2), intralysosomal iron chelation completely protected the c

19 iven by reactive oxygen species (ROS), is an intralysosomal, non-degradable, auto-fluorescent macromo

20 ed to lysosomal nucleoside buildup, elevated intralysosomal pH, and altered macrophage function.

21 This is due to an elevation of the intralysosomal pH, since ionophores and proton pump inhi

22 P-dependent proton pump V-ATPase ensures low intralysosomal pH, which is essential for lysosomal hydr

23 NPC2 is an intralysosomal protein that binds cholesterol in vitro.

24 NPC2 is a small intralysosomal protein that has been characterized bioch

25 hese results indicate that the generation of intralysosomal ROS induces lysosomal membrane permeabili

26 somes and proteolytically processed, but its intralysosomal stability is intermediate between that of

27 al neurodegenerative disease presenting with intralysosomal storage, enhances clearance of proteolipi

28 ion of GM2AP is to bind and extract GM2 from intralysosomal vesicles, forming a soluble protein-lipid

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