Results 71 to 80 of about 6,268,347 (286)

Lysosomal Storage Diseases

Journal of Inborn Errors of Metabolism and Screening, 2014
Lysosomal storage diseases are a group of inherited and acquired disorders. They are characterized by interruption of recycling of cellular and extracellular molecules. Clinically, they are presented as developmental and neurological symptoms similar to other inherited and acquired disorders.
Alroy, Joseph, Lyons, Jeremiah A.
openaire   +4 more sources

Targeting CD300a Signaling With a Phosphatidylserine‐Presenting Hydrogel Rescues Macrophage Dysfunction and Accelerates Diabetic Wound Healing

Advanced Healthcare Materials, EarlyView.
Schematic illustration of the proposed mechanism: PEG/RGD‐PSLs mimic apoptotic cells to engage PS receptors (notably CD300a), transducing an inhibitory signal that suppresses the MyD88/NF‐κB pathway, leading to global anti‐inflammatory and pro‐reparative effects.
Lele Wu, Xuetong Hao, Yang Zou, Xinglin Chen, Junyan Kong, Chunning Gu, Wenjie Ma, Zhongjun Yang, Huifen Liu, Mengmeng Liu, Xin Tong   +10 more
wiley   +1 more source

Engineered Nanoplatforms Targeting Activated Hepatic Stellate Cells for Liver Fibrosis Therapy via TGF‐β Axis Inhibition and Ferroptosis Induction

Advanced Healthcare Materials, EarlyView.
FeS‐VA@lanifibranor (FVL), an activated hepatic stellate cell (aHSC)‐targeted nanoplatform comprising vitamin A‐functionalized two‐dimensional iron sulfide nanosheets and lanifibranor, is engineered for precision therapy of liver fibrosis. FVL suppresses the TGF‐β signaling pathway by inhibiting SMAD2/3 phosphorylation and induces ferroptosis in aHSCs,
Yinuo Yang, Mengyao Mu, Ke Ren, Hui Liu, Pengju Ma, Yingqi Wang, Mengmeng Zhang, Xueli Xu, Wenzhe Li, Wenshuang Gao, Xiao Sun, Qiang Zhu   +11 more
wiley   +1 more source

A Novel Retinal Gene Therapy Strategy for Batten Disease and Beyond

Proceedings, 2020
Batten Disease is a fatal lysosomal storage disorder characterized by cognitive and [...]
Maura Schwartz, Alex Campbell, Joseph Caporale, Megan Baird, Isabella Palazzo, Shibi Likhite, Andrew Fischer, Kathrin Meyer   +7 more
doaj   +1 more source

Loss of Niemann-Pick C1 or C2 protein results in similar biochemical changes suggesting that these proteins function in a common lysosomal pathway. [PDF]

PLoS ONE, 2011
Niemann-Pick Type C (NPC) disease is a lysosomal storage disorder characterized by accumulation of unesterified cholesterol and other lipids in the endolysosomal system.
Sayali S Dixit, Michel Jadot, Istvan Sohar, David E Sleat, Ann M Stock, Peter Lobel   +5 more
doaj   +1 more source

Optical Detection of Cellular Signals at Material Interfaces

Advanced Healthcare Materials, EarlyView.
Emerging functional materials are transforming optical detection of cellular signals. This review highlights optical techniques that exploit the unique optical properties of diverse materials to detect and quantify cellular electrical, chemical, and mechanical signals, and discusses key opportunities and challenges.
Xuchen Ren, Jitong Ren, Zixin Zhang, Koney Eungkon Kim, Francis L. Hong, Yuecheng Peter Zhou   +5 more
wiley   +1 more source

Tau accumulation in degradative organelles is associated to lysosomal stress

Scientific Reports, 2023
Neurodegenerative disorders are characterized by the brain deposition of insoluble amyloidogenic proteins, such as α-synuclein or Tau, and the concomitant deterioration of cell functions such as the autophagy-lysosomal pathway (ALP).
Ester Piovesana, Claudia Magrin, Matteo Ciccaldo, Martina Sola, Manolo Bellotto, Maurizio Molinari, Stéphanie Papin, Paolo Paganetti   +7 more
doaj   +1 more source

Lysosomal storage disease : revealing lysosomal function and physiology

, 2010
The discovery over five decades ago of the lysosome, as a degradative organelle and its dysfunction in lysosomal storage disorder patients, was both insightful and simple in concept.
Parkinson-Lawrence, E., Borlace, G., Prodoehl, M., Shandala, T., Plew, R., Brooks, D.   +5 more
core   +1 more source

Chemical Strategies for Multistimuli Responsive Dynamic Covalent Materials in Regenerative Medicine

Advanced Healthcare Materials, EarlyView.
Multistimuli responsive dynamic covalent materials integrate reversible chemistry with the complex chemical, mechanical, and biochemical cues of regenerative environments. This review highlights chemical strategies based on orthogonal dynamic bonds, multicomponent networks, and hierarchical architectures to regulate mechanics, degradation, and ...
Saurabh Joshi, Matthew B. Baker, Dominik Schauenburg   +2 more
wiley   +1 more source

Chronic intestinal pseudo-obstruction. Did you search for lysosomal storage diseases?

Molecular Genetics and Metabolism Reports, 2017
Chronic intestinal pseudo-obstruction results in clinical manifestations that resemble intestinal obstruction but in the absence of any physical obstructive process.
J. Politei, C. Durand, A.B. Schenone, A. Torres, J. Mukdsi, B.L. Thurberg   +5 more
doaj   +1 more source