The Metabolism of Tay-Sachs Ganglioside: Catabolic Studies with Lysosomal Enzymes
from Normal and Tay-Sachs Brain Tissue, Journal of Clinical Investigations, 51: 2339-2345, 1972.
Direct multiplex assay of lysosomal enzymes
in dried blood spots for newborn screening.
Furthermore, quercetin supplementation modulated lysosomal enzyme
activities in various diseases [18,19] suggesting that the hepatoprotective effect of quercetin against alcohol-derived injury might be involved in the regulation and chelation of quercetin on lysosomal labile iron.
Zuhlsdorf M, Imort M, Hasilik A, von Figura K (1983) Molecular forms of beta-hexosaminidase and cathepsin D in serum and urine of healthy subjects and patients with elevated activity of lysosomal enzymes
All lysosomal enzymes
have mannose 6-phosphate on the nonreducing termini of their high mannose-type glycans, synthesized by GlcNAc-1-phosphotransferase and [alpha]-N-acetylglucosaminidase (uncovering enzyme) localized in the cis-Golgi and trans-Golgi network, respectively.
J et al ; Nonsteroidal anti inflammatory agents; Regulation of phagocytic secretion of lysosomal enzymes
from guinea pig neutrophils; Jl of Rheumatology and experimental therapeutics;1978; 207: 618-629
Furthermore, further lipopoly-saccharide-induced activation of the Kupffer cells, may contribute to the late liver dysfunction and to the increased synthesis of the lysosomal enzymes
such as GLU .
Without these important recognition markers, lysosomal enzymes
are not transported to the lysosomes; instead, they leak out of cells, and the serum levels of these enzymes are higher than normal.
It is caused by a lack of lysosomal enzymes
, which are needed to break down long chains of sugar carbohydrates in each of our cells that help build bone, cartilage, tendons, corneas, skin and connective tissue.
3,4) There are reports on the blood and tissue levels of lysosomal enzymes
Large Scale Biology Corporation (Vacaville, CA) has patented galactosidase truncated at the carboxy terminus and the production of enzymatically active recombinant human and animal lysosomal enzymes
involving construction and expression of recombinant expression constructs comprising coding sequences of human or animal lysosomal enzymes
in a plant expression system.
Dapsone (4,4' diaminodiphenylsulfone) inhibits neutrophil migration and endothelial attachment, and it blocks neutrophil secretion of lysosomal enzymes
Endosomes then fuse with lysosomes so that the lysosomal enzymes
gain access to the ingested material.
The neuronal albumin uptake and other changes described would seem to indicate serious neuronal damage, which may be mediated through organelle damage with release of not only hydrolytic lysosomal enzymes
but also, for example, sequestered harmful material, such as heavy metals, stored away in cytoplasmic organelles (lysosomes).
When neutrophils are specifically triggered by any one of the proinflammatory signals, a number of cytotoxic factors are released during respiratory burst, such as oxygen free radicals, lysosomal enzymes
, cytokines, and arachidonic acid metabolites.