The mucopolysaccharides, which aid in bone formation, as well as that of the skin, cornea and cartilage, don't perform their function properly because of a lack
of lysosomal enzymes.
The disease causes an accumulation of mucopolysaccharides, or glycosaminoglycans (GAGs) due to the impaired functioning
of lysosomal enzymes that break down GAGs which help build bones, cartilage, skin, tendons, corneas and the fluid responsible for lubricating joints.
The ensuing decrease in pH and / or the presence
of the lysosomal enzymes such as cathepsin B lead to premature activation of trypsinogen.
LTB4 increases edema and chemotaxis, induces release
of lysosomal enzymes, increases reactive oxygen species, and enhances production of the cytokines TNF - α, IL - 1, and IL - 6.»
Comparative binding, endocytosis, and biodistribution of antibodies and antibody - coated carriers for targeted delivery
of lysosomal enzymes to ICAM - 1 versus transferrin receptor.
Not exact matches
Specifically, the current study demonstrated that an accumulation
of oxidized dopamine depressed the activity
of lysosomal glucocerebrosidase (GCase), an
enzyme implicated in PD.
Targeting, Endocytosis, and
Lysosomal Delivery
of Active
Enzymes to Model Human Neurons by ICAM -1-Targeted Nanocarriers.
So the idea would be to identify the
enzymes these organisms use to digest
lysosomal wastes, modify them a bit to help them work in the slightly different environment
of the human lysosome, and then deliver them to where they need to go in our cells.
If such
enzymes didn't exist, then the planet would be ankle - deep in the undegraded
lysosomal wastes left over from the cells
of 600 million years
of animal life on this planet.
Our lead candidate, LYS - GM101, has been designed to replace this defective gene in the cells
of GM1 patients, in order to allow for production
of the functional
lysosomal acid beta - galactosidase (bgal)
enzyme.
Furthermore, ApoE4 caused a disruption
of lysosomal integrity and a shift in the localization
of Cathepsin D, an
enzyme known to degrade Trx1.
Lysis
of these A beta 42 - burdened neurones apparently resulted in a local, radial dispersion
of their cytoplasmic contents, including A beta 42 and
lysosomal enzymes, into the surrounding extracellular space.
Quantitative Differences in the Urinary Proteome
of Siblings Discordant for Type 1 Diabetes Include
Lysosomal Enzymes.
Cutting Edge: Induction
of the Antigen - Processing
Enzyme IFN --LCB- gamma -RCB-- Inducible
Lysosomal Thiol Reductase in Melanoma Cells Is STAT1 - Dependent but CIITA - Independent
However, data from other animal models demonstrate diminished
lysosomal enzyme activity during extended periods
of energy restriction (39).
Vacuole formation is thought to be a consequence
of the uncoupling
of exocytosis
of zymogens and abnormal intracellular traffi cking
of digestive and
lysosomal enzymes.
Biochemical studies have shown that these vacuoles are the product
of co-localization
of zymogens
of digestive
enzymes and
lysosomal enzymes, which are normally strictly segregated.
In the lipid storage form
of lysosomal storage disease I mentioned that dogs and cats can be born with decreased or missing lysosome
enzymes that allows them to process fatty substances (lipids).
For example, the conversion
of trypsinogen (inactive) to its active form, trypsin, can be triggered by enterokinase, bile,
lysosomal enzymes or other stimuli.