Sentences with phrase «inodify lysosomal»

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.

They chose Niemann - Pick Type C, a lysosomal storage disease associated with mutations in two ancient genes that are well - conserved in many organisms.

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.

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

In another study representing the first time precision medicine has been applied to feline health, Lyons and her team used whole genome sequencing and the 99 Lives consortium to identify a lysosomal disorder in a 36 - week - old silver tabby kitten that was referred to the MU Veterinary Health Center.

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.

Early - stage data suggests the investigative LAMP - Vax technology works by fusing tumor antigens to a cellular protein known as LAMP (Lysosomal - Associated Membrane Protein).

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.

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.

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.

Twelve percent encoded mitochondrial proteins such as succinate dehydrogenase complex, subunit B, iron sulfur (Ip)(τ statistic = — 0.76, P = 2.2 × 10 — 7), and ubiquinol — cytochrome c reductase subunit (τ statistic = — 0.65, P = 7.4 × 10 — 6)(Figure 1), and 6 % encoded lysosomal proteins (Supplemental Table 2, http://www.jci.org/cgi/content/full/112/12/1796/DC1).

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.

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.

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

The α - arrestin ARRDC3 mediates ALIX ubiquitination and G protein — coupled receptor lysosomal sorting.

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

High - fidelity hydrophilic probe for two - photon fluorescence lysosomal imaging.

The small chemical vacuolin - 1 inhibits Ca2 & plus; - dependent lysosomal exocytosis but not cell resealing.

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

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

AP - 3 regulates PAR1 ubiquitin - independent MVB / lysosomal sorting via an ALIX - mediated pathway.

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.

When an LDL particle (carrying cholesterol) binds to the LDLR, the particle is trafficked into the hepatocyte, and PCSK9 targets the receptor for lysosomal degradation.

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

SPE - 39 family proteins interact with the HOPS complex and function in lysosomal delivery.

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

This contraction was dependent on lysosomal, but not the sarcoplasmic reticulum Ca2 + stores.

Neuraminidase 1 is a negative regulator of lysosomal exocytosis.

The frontotemporal lobar degeneration risk factor, TMEM106B, regulates lysosomal morphology and function.

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.

Indeed, mutations in multiple lysosomal genes result in severe neurodegeneration and it is becoming more clear that lysosomes also play a neuroprotective role in more common neurodegenerative diseases such as Alzheimer's disease that have not traditionally been associated with lysosomal defects.

Prosaposin facilitates sortilin - independent lysosomal trafficking of progranulin.

Lysosomal NEU1 deficiency affects amyloid precursor protein levels and amyloid - β secretion via deregulated lysosomal exocytosis.

Donor - Acceptor - Donor Fluorene Derivatives for Two - Photon Fluorescence Lysosomal Imaging.

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.

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.

... [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.

Excessive lysosomal exocytosis is not a good thing in many instances.

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.

New research indicates that placental stem cell engraftment may represent an interesting new strategy to treat an enzyme deficiency associated with a lysosomal storage disorder