Lysosomal enzymes are less stable when fibroblast sonicates are incubated at neutral pH. By contrast, non-lysosomal enzymes and the majority of cell proteins are resistant to degradation at neutral pH. These data indicate that the low pH of the lysosomes serves two purposes: (1) to provide an...
4C). In summary, sCI-MPR purified from bovine serum is active and capable of binding IGF2 and phosphomannosyl-containing lysosomal enzymes GAA and PPT1 with high affinity. Fig. 4 Binding affinity at pH 7.4 of IGF2 and the lysosomal enzymes GAA and PPT1 to sCI-MPR as assessed by SPR. ...
The majority of LSDs are caused by deficient or defective activity of glycosidases. The natural substrates of these enzymes comprise a wide array of glycoconjugates, many of which are still unidentified, that are themselves differentially expressed and distributed in different cell types. Genetic mutat...
This mechanism would help ensure that only N-linked glycans of lysosomal enzymes are phosphorylated.This is a preview of subscription content, access via your institution Access optionsAccess Nature and 54 other Nature Portfolio journals Get Nature+, our best-value online-access subscription 24,99...
Conversely, CLN3 overexpression promotes the formation of multiple lysosomal tubules, which are autophagy and CI-M6PR-dependent, generating newly formed proto-lysosomes. Together, our findings reveal that CLN3 functions as a link between the M6P-dependent trafficking of lysosomal enzymes and lysosomal...
[7,8]. However, because both macroautophagy and CMA are dependent on intact lysosomes, enhancing macroautophagy may not be effective in clearing potentially neurotoxic proteins if lysosomal function is impaired. Understanding the role of lysosomal enzymes in α-syn clearance may provide new strategies...
Since lysosomes are increased in osteoarthritis, it is possible that the abnormal biosynthetic patterns exhibited by cells in areas of degeneration are caused by such enzymes. Articular cartilage is an avascular tissue with very low cell density, composed primarily of extracellular substances such as ...
Activities of lysosomal enzymes acid phosphatase, N-acetyl-beta-D-glucosaminidase, beta-galactosidase, acid lipase and cathepsins B and D were studied after accumulation of neutral red, acridine orange chloroquine and daunorubicin in lysosomes of fibroblasts of the LSM substrain. All the drugs studied...
S3i), together indicating that the stable structure of lysoTRAP restricted the release of ACE2 and enzymes. Consequently, over 80% of the ACE2, protease and RNase enzymatic activities in lysoTRAP were retained after 72 h incubation at 37 °C (Fig. 1k), suggesting the stable biological...
Dysfunction in some of these proteins lead to lysosomal storage disorders (LSDs), which collectively have a relatively high incidence in the general population: more than 1:5000 live births are affected by a LSD2. Notable among these lysosomal enzymes is β-glucocerebrosidase (GCase), which is...