It's a metabolic storage disorder resulting
in lysosomal enzyme deficiency, which affects nearly every system in the dog's body.
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.
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
The ensuing decrease
in pH and / or the presence of the
lysosomal enzymes such as cathepsin B lead to premature activation of trypsinogen.
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.
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).
It is difficult to treat abscesses
in guinea pigs, because like rabbits, they lack a
lysosomal enzyme that helps to break down pus.