"Lysosomal" refers to something related to lysosomes, which are special compartments in our cells that work as recycling centers. They break down and recycle waste materials, like broken cell parts or old molecules, to keep the cell healthy. Therefore, when we use the word
"lysosomal," we are talking about something that has to do with this process of breaking down and recycling within our cells.
Full definition
An early onset form of
lysosomal storage disease, this can cause affected dogs to display neurologic signs as puppies or young adults.
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.
It's a metabolic storage disorder resulting in
lysosomal enzyme deficiency, which affects nearly every system in the dog's body.
D'Oro, U., Vacchio, M.S., Weissman, A.M. & Ashwell, J.D. Activation of the Lck tyrosine kinase targets cell surface T cell antigen receptors for
lysosomal degradation.
Elizabeth B. Moloney, PhD, is a post-doctoral research fellow at the Neuroregeneration Research Institute at McLean Hospital, where she is involved in developing both in vitro and in vivo approaches for understanding the role of
lysosomal function in the pathophysiology of cellular aging and...
«The mitochondrial and
lysosomal pathways are two critical pathways in disease development,» said Krainc, who also is the director of the Center for Rare Neurological Diseases and a professor of neurological surgery and of physiology.
A closely related protein, beta - synuclein, typically does not form fibrils in the cytoplasm but appears to be involved in
lysosomal dysfunction in some forms of dementia.
D'Azzo's long - standing interest in sialidosis and related disorders known
as lysosomal storage diseases led to the discovery.
For the first time ever, the present study showed that AMD is associated with impaired
lysosomal autophagy, which is an important clean - up mechanism of the fundus of the eye.
When this process starts, the damaged muscle fibers are broken down by
lysosomal proteases, free radicals and many other compounds released by the macrophages.
Increased LAMP1 levels were followed by
excessive lysosomal exocytosis in nerve cells, resulting in increased release of Alzheimer - linked peptides from neurons.
What affect does remofusin have
on lysosomal pH in RPE and non-RPE cells and can this affect the normal turn - over of lysosomally - targeted proteins and organelles?
Our clinic manages patients
with lysosomal storage diseases and I personally manage patients with Fabry and Pompe disease.
Quantitative Differences in the Urinary Proteome of Siblings Discordant for Type 1 Diabetes
Include Lysosomal Enzymes.
Here we show that
lysosomal pathology in AD brain involves extensive alterations of macroautophagy, an inducible pathway for the turnover of intracellular constituents, including organelles.
The molecular mechanism of autophagy and its relationship to
other lysosomal degradation pathways remain incompletely understood.
PV - 10 (rose bengal disodium 10 %), a small - molecule fluorescein derivative, while excluded from normal cells, transits the plasmalemma of cancer cells and accumulates in lysosomes, triggering
lysosomal release and complete autolysis within 30 to 60 minutes.
«The goal of this study was to determine the effects of mechanical ventilation - induced activation of the autophagy /
lysosomal system on ventilator - induced diaphragm mitochondrial dysfunction,» Smuder said.
Increased
lysosomal activity has been demonstrated in response to short - term fasting in transgenic mice expressing the autophagy gene, microtubule - associated protein 1A / 1B - light chain 3, fused with green fluorescent protein (38).
One of these compounds enabled chloride transport in real cells without seriously affecting
lysosomal pH.
AA407268; AA407316; Aglp; Aglp; Apl; Apl; Bat - 7; Bat - 7; Bat7; Bat7; G9; HLA - B - associated transcript 7; Map - 2; Map - 2; NANH; NEU; Neu - 1; Neu - 1; SIAL1; acid phosphatase, liver; alpha glucosidase processing; expressed sequence AA407268; expressed sequence AA407316;
lysosomal sialidase; mannosidase processing 2; sialidase 1
I would like to know the mechanism by which the drug is
triggering lysosomal exocytosis and if it is only occurring in RPE cells?
GM1 gangliosidosis, or Landing disease, is a rare inherited
neurodegenerative lysosomal storage disorder characterized by severe cognitive and motor developmental delays resulting in the death of most patients at a very young age.
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.
His research focuses on the molecular therapy of neurodegenerative diseases, specifically the therapeutic strategies for the
neuropathic lysosomal storage diseases known as GM1 and GM2 Gangliosidosis in cats.
Fucosidosis is an autosomal - recessive
genetic lysosomal storage disease of the English Springer Spaniel dog that is progressive and invariably fatal.
The research establishes a new model for
studying lysosomal storage disorders and suggests that GM2 accumulation not only causes neurodegeneration but also may disrupt neurodevelopment prior to birth.
Another example of a transceptor is SLC38A9, which
mediates lysosomal arginine signalling [14,15], but also has transport function.
Patients with mucolipidosis type IV, an autosomal
recessive lysosomal storage disease, may be constitutively achlorhydric.
The drug candidate is intended for the treatment of subjects with neuronal ceroid lipofuscinosis, aka Batten Disease, a
fatal lysosomal storage disease that primarily affects the nervous system in children.
Last month researchers led by Thomas Kukar, a professor at Emory University, published a paper showing that trehalose can
reverse lysosomal deficiencies in mouse models of frontotemporal dementia.
Cuervo's group found that a mutant protein associated with a heritable version of Parkinson's gums
up lysosomal channels.
Although the vesicles exclude
soluble lysosomal contents and antigen - processing machinery, many contain MHC I and B7 costimulatory molecules.
Specifically, the current study demonstrated that an accumulation of oxidized dopamine depressed the activity of
lysosomal glucocerebrosidase (GCase), an enzyme implicated in PD.
That depression in turn weakened
overall lysosomal function and contributed to degeneration of neurons.
These actions are specific for the sympathetic and parasympathetic neurotransmitters and for the two cyclic nucleotides, as phenylephrine, tyramine, choline, adenosine 5» - monophosphate and guanosine 5» - monophosphate do not
inodify lysosomal enzyme release.