Results 101 to 110 of about 8,108 (242)

A mouse model for fucosidosis recapitulates storage pathology and neurological features of the milder form of the human disease

Disease Models & Mechanisms, 2016
Fucosidosis is a rare lysosomal storage disorder caused by the inherited deficiency of the lysosomal hydrolase α-L-fucosidase, which leads to an impaired degradation of fucosylated glycoconjugates.
Heike Wolf, Markus Damme, Stijn Stroobants, Rudi D'Hooge, Hans Christian Beck, Irm Hermans-Borgmeyer, Renate Lüllmann-Rauch, Thomas Dierks, Torben Lübke   +8 more
doaj   +1 more source

Bioadhesive Hydrogel With Polyphenol‐Armored Nanogene Rejuvenates Chondrocyte Senescence for Aged Osteoarthritis Therapy

Advanced Science, EarlyView.
An adhesive hydrogel depot with polyphenol‐armored nanogenes is developed for treating aged osteoarthritis. This system maintains miR‐140 bioactivity, prolongs its intra‐articular retention, and enhances transfection into chondrocytes, leading to the downregulation of senescence‐associated pathways.
Liwei Yan, Runze Yang, Ting Zhou, Tianhao Xu, Yongqi Li, Yuelin Hu, Minghao Ge, Lei Zhang, Xiong Lu, Chaoming Xie, Weili Fu   +10 more
wiley   +1 more source

Dual Physiological Barriers Bypassed by a Silk‐Based Supramolecular Protein Delivery Platform for Neuroinflammation Mitigation in Alzheimer's Disease

Advanced Science, EarlyView.
A supramolecular nanocomplex based on phenolic‐modified silk sericin is developed to deliver antioxidant enzymes across the blood–brain barrier and escape lysosomal degradation. This dual‐barrier‐crossing system reduces neuroinflammation and improves cognitive performance in Alzheimer's disease mouse model, offering a promising strategy for protein ...
Doudou Hu, Tiandong Li, Jian He, Yujian Jiang, Yeyuan Wang, Jingchen Sun   +5 more
wiley   +1 more source

Targeting Endogenous Lipophagy: A Novel Strategy to Enhance MSC Osteogenesis and Mineralization for Senile Osteoporosis Therapy

Advanced Science, EarlyView.
Schematic representation of the role of lipophagy in bone mesenchymal stem cells(MSCs). In healthy MSCs, functional lipophagy efficiently degrades lipid droplets to support oxidative phosphorylation and cellular energy production, thereby facilitating osteogenic differentiation and matrix mineralization.
Chaoqiang Chen, Zhidong Liu, Yanhang Sun, Xiaojun Xu, Zhexiao Lan, Junhao Zhao, Peitao Xu, Guiwen Ye, Jinteng Li   +8 more
wiley   +1 more source

Lysosomal storage disease 2 - Pompe's disease

, 2008
Pompe's disease, glycogen-storage disease type II, and acid maltase deficiency are alternative names for the same metabolic disorder. It is a pan-ethnic autosomal recessive trait characterised by acid alpha-glucosidase deficiency leading to lysosomal ...
van der Ploeg, Ans T., Reuser, Arnold J. J.   +1 more
core   +1 more source

Scalable Engineering of Bio‐Manufactured Extracellular Vesicles for Selective Delivery in Ovarian Cancer Patient‐Derived Models

Advanced Science, EarlyView.
Engineered extracellular vesicles displaying Ephrin‐B2 selectively target Ephrin‐B4–expressing ovarian cancer cells, enabling precise delivery in patient‐derived models. This scalable bio‐manufacturing platform reveals a versatile strategy to exploit Ephrin signaling for highly specific therapeutic payload delivery and motivates exploration of tailored
Nihar Godbole, Andrew Lai, Alexander Quinn, Marianne Gillard, Dominic Guanzon, Katherin Scholz‐Romero, Alexis Salas‐Burgos, Bastián Lillo Dapremont, Rohan Lourie, Naven Chetty, Richard Lobb, Kate Beecher, Amy E. McCart Reed, Kaltin Ferguson, Biao Sun, Yaowu He, John D. Hooper, Carlos Salomon   +17 more
wiley   +1 more source

Galactosialidosis in two siblings

The Turkish Journal of Pediatrics, 1996
Galactosialidosis is a rare lysosomal storage disease associated with deficiencies of alpha-galactosidase and beta-neurominidase. In this report, two siblings with galactosialidosis, resembling Niemann-Pick disease with the presence of foamy cells
A Yuce, N Kocak, G T Besley
doaj  

Fluorogenic Substrates for Visualizing Acidic Organelle Enzyme Activities.

PLoS ONE, 2016
Lysosomes are acidic cytoplasmic organelles that are present in all nucleated mammalian cells and are involved in a variety of cellular processes including repair of the plasma membrane, defense against pathogens, cholesterol homeostasis, bone remodeling,
Fiona Karen Harlan, Jason Scott Lusk, Breanna Michelle Mohr, Anthony Peter Guzikowski, Robert Hardy Batchelor, Ying Jiang, John Joseph Naleway   +6 more
doaj   +1 more source

Modelling the neuropathology of lysosomal storage disorders through disease-specific human induced pluripotent stem cells [PDF]

, 2019
Mucopolysaccharidosis II (MPS II) is a lysosomal storage disorder (LSD), caused by iduronate 2-sulphatase (IDS) enzyme dysfunction. The neuropathology of the disease is not well understood, although the neural symptoms are currently incurable.
Nakanishi, H., Westerveld, Michael, Laszlo, L., Lo Giudice, Maria, László, L, Pijnappel, WWM Pim, Varga, Norbert, Kobolák, J, Kitiyanant, N, Hyttel, Poul, Bock, István, Jezsó, B, Hyttel, P., Zhou, S, Pijnappel, Pim, Jerso, B., Zhou, SL, Téglási, A, Varga, Eszter, Jerso, B, Pijnappel, WWM P, Dinnyés, A, Pijnappel, W., Dinnyés, András, Varga, N., Freude, K., Freude, K, Hyttel, P, Westerveld, M. (Michael), Phanthong, P., Nakanishi, H, Molnar, K., Dinnyes, A., Nakanishi, Hideyuki, Lo Giudice, M, Bock, I., Bock, I, Kobolák, Julianna, Hoogeveen-Westerveld, M, Hoogeveen - Westerveld, Marianne, Kitiyanant, Narisorn, Varga, E, Freude, Kristine, Phanthong, Phetcharat, Kitiyanant, N., Varga, N, Varga, E. (Endre), Teglasi, A., Jezsó, Bálint, Molnár, Kinga, Molnár, K, Varga, Endre, László, Lajos, Kobolak, J., Zhou, Shuling, Phanthong, P, Lo Giudice, M., Hoogeveen-Westerveld, Marianne, Zhou, S.L., Téglási, Annamária   +59 more
core   +1 more source

Light‐Switched Mesenchymal Stem Cells for In Situ Exosome Amplification in Craniofacial Bone Defect Reconstruction

Advanced Science, EarlyView.
Light‐switchable MSCs (MSC‐UCNPs) were constructed by intracellular incorporation of UCNPs. Upon 980 nm irradiation, UCNPs emitted localized ultraviolet light (365 nm), activating the ROS/HEXB/LAMP1 signaling pathway to suppress lysosome–multivesicular body fusion and thereby enhance exosome biogenesis. Embedded within an injectable hydrogel, MSC‐UCNPs
Tingting Wu, Yajing Liu, Shuman Wang, Xiaoming Bai, Luyun Zhang, Yuwei Liu, Zhiwen Fu, Chen Shi   +7 more
wiley   +1 more source