Sentences with phrase «of lysosomal»

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).

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.

A type of lysosomal storage disease, this can cause skeletal abnormalities, growth retardation, and gait abnormalities, and can require close monitoring and special measu...

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.

Lipidosis: a form of lysosomal storage disease where lipids accumulate in nerves.

An early onset form of lysosomal storage disease, this can cause affected dogs to display neurologic signs as puppies or young adults.

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.»

Type 1 Diabetes: Urinary Proteomics and Protein Network Analysis Support Perturbation of Lysosomal Function.

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 protein is a key regulator of lysosomal exocytosis.

Furthermore, ApoE4 caused a disruption of lysosomal integrity and a shift in the localization of Cathepsin D, an enzyme known to degrade Trx1.

A point mutation in Neu1 is responsible for a partial deficiency of lysosomal neuraminadase and may explain the altered immune response (Rottier et al. 1998).

... [For example], A deficiency of lysosomal sialidase (Neu1) leads to increased levels of LAMP -1-mediated lysosomal exocytosis by neutrophils in the bone marrow cavity.

Many of the lysosomal proteases are active at neutral pH and this would mean that they could potentially cleave cell surface receptors and cell adhesion molecules.

Neuraminidase 1 is a negative regulator of lysosomal exocytosis.

Comparative binding, endocytosis, and biodistribution of antibodies and antibody - coated carriers for targeted delivery of lysosomal enzymes to ICAM - 1 versus transferrin receptor.

These are critical observations that will allow the field of autophagy to move beyond the utilization of lysosomal inhibitors for the pharmacological treatment of cancer by targeting autophagy.

The researchers found that degenerative changes and loss of vision are caused by impaired function of the lysosomal clean - up mechanism, or autophagy, in the fundus of the eye.

Specifically, the current study demonstrated that an accumulation of oxidized dopamine depressed the activity of lysosomal glucocerebrosidase (GCase), an enzyme implicated in PD.

Not exact matches

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.

Wnt proteins, regulators of development in many organisms, bind to seven transmembrane — spanning (7TMS) receptors called frizzleds, thereby recruiting the cytoplasmic molecule dishevelled (Dvl) to the plasma membrane.Frizzled - mediated endocytosis of Wg (a Drosophila Wnt protein) and lysosomal degradation may regulate the formation of morphogen gradients.

Cuervo's group found that a mutant protein associated with a heritable version of Parkinson's gums up lysosomal channels.

Functional analyses indicate that MFSD12 encodes a lysosomal protein that affects melanogenesis in zebrafish and mice, and that mutations in melanocyte - specific regulatory regions near DDB1 / TMEM138 correlate with expression of ultraviolet response genes under selection in Eurasians.

«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.

That depression in turn weakened overall lysosomal function and contributed to degeneration of neurons.

Genzyme has long been known for our expertise in the class of rare genetic diseases known as lysosomal storage disorders (LSDs).

One of these compounds enabled chloride transport in real cells without seriously affecting lysosomal pH.

The authors also employed other advanced imaging techniques — including electron microscopy and super-resolution imaging — to discover that the formation, and subsequent loosening, of these contacts is regulated by a lysosomal protein called RAB7.

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.

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.

The molecular mechanism of autophagy and its relationship to other lysosomal degradation pathways remain incompletely understood.

Members of the section use the NIH Clinical Center to evaluate individuals with cystinosis, a lysosomal membrane defect leading to storage of the amino acid cystine and causing kidney failure in childhood.

Rapid and reversible knockdown of endogenous proteins by peptide - directed lysosomal degradation.

Lysosomal sequestration of sunitinib: a novel mechanism of drug resistance.

Enhanced delivery of α - glucosidase for Pompe disease by ICAM -1-targeted nanocarriers: comparative performance of a strategy for three distinct lysosomal storage disorders.

Sucrose - induced vacuolation results in increased expression of cholesterol biosynthesis and lysosomal genes.

ALIX Regulates the Ubiquitin - Independent Lysosomal Sorting of the P2Y 1 Purinergic Receptor via a YPX 3 L Motif.

Targeting, Endocytosis, and Lysosomal Delivery of Active Enzymes to Model Human Neurons by ICAM -1-Targeted Nanocarriers.

Deficiency of ATP13A2 leads to lysosomal dysfunction, α - synuclein accumulation, and neurotoxicity.

CXCR4 may then be recycled to the cell surface or ubiquitinated by AIP4 that results in the lysosomal degradation of CXCR4.

Building on these extreme examples that emphasize the potential roles played by healthy lysosomes in protecting neurons against the age - dependent accumulation of toxic cellular debris, we ultimately seek to understand how neurons sense and regulate the status of their lysosomes, how lysosomes are affected during age - related disease states and whether lysosomal function can be modulated for therapeutic purposes.

Prosaposin facilitates sortilin - independent lysosomal trafficking of progranulin.

This is my postdoctoral work from St Jude Children's Hopsital in Sandra d'Azzo's lab and is an exampe of excessive lysosomal exocytosis being pathological.

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.

It is one of over 50 genetically inherited disorders known as Lysosomal Storage Diseases.

In Neu1 - deficient mice the excessive lysosomal exocytosis results in cleavage of VCAM - 1, loss of bone marrow progenitor cells from the bone niche and the onset of spleenic hematopoiesis.

We further demonstrate that lysosomal exocytosis mediates the resealing of primary skin fibroblasts wounded during the contraction of collagen matrices.

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.

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.