Results 21 to 30 of about 2,448 (132)

Neuronal ceroid lipofuscinoses in children

Annals of Indian Academy of Neurology, 2021
Background: The neuronal ceroid lipofuscinoses (NCL) constitute a group of gray matter neurodegenerative disorders characterized by the accumulation of ceroid lipopigment in lysosomes in neurons and other cell types.
Mahesh Kamate, Narendranadha Reddy, Mayank Detroja, Virupaxi Hattiholi   +3 more
doaj   +1 more source

Shotgun Proteomics of Isolated Urinary Extracellular Vesicles for Investigating Respiratory Impedance in Healthy Preschoolers

Molecules, 2021
Urine proteomic applications in children suggested their potential in discriminating between healthy subjects from those with respiratory diseases.
Giuliana Ferrante, Rossana Rossi, Giovanna Cilluffo, Dario Di Silvestre, Andrea Brambilla, Antonella De Palma, Chiara Villa, Velia Malizia, Rosalia Gagliardo, Yvan Torrente, Giovanni Corsello, Giovanni Viegi, Pierluigi Mauri, Stefania La Grutta   +13 more
doaj   +1 more source

Extracellular Vesicles Released by Genetically Modified Macrophages Activate Autophagy and Produce Potent Neuroprotection in Mouse Model of Lysosomal Storage Disorder, Batten Disease

Cells, 2023
Over the recent decades, the use of extracellular vesicles (EVs) has attracted considerable attention. Herein, we report the development of a novel EV-based drug delivery system for the transport of the lysosomal enzyme tripeptidyl peptidase-1 (TPP1) to ...
Nazira El-Hage, Matthew J. Haney, Yuling Zhao, Myosotys Rodriguez, Zhanhong Wu, Mori Liu, Carson J. Swain, Hong Yuan, Elena V. Batrakova   +8 more
doaj   +1 more source

Tripeptidyl Peptidase II Promotes Maturation of Caspase-1 inShigella flexneri-Induced Macrophage Apoptosis [PDF]

Infection and Immunity, 2000
ABSTRACTThe invasive enteropathogenic bacteriumShigella flexneriactivates apoptosis in macrophages.Shigella-induced apoptosis requires caspase-1. We demonstrate here that tripeptidyl peptidase II (TPPII), a cytoplasmic, high-molecular-weight protease, participates in the apoptotic pathway triggered byShigella.
H, Hilbi, R J, Puro, A, Zychlinsky
openaire   +2 more sources

A High-Content Screen Identifies TPP1 and Aurora B as Regulators of Axonal Mitochondrial Transport

Cell Reports, 2019
Summary: Dysregulated axonal trafficking of mitochondria is linked to neurodegenerative disorders. We report a high-content screen for small-molecule regulators of the axonal transport of mitochondria.
Evgeny Shlevkov, Himanish Basu, Mark-Anthony Bray, Zheng Sun, Wei Wei, Kaan Apaydin, Kyle Karhohs, Pin-Fang Chen, Janell L.M. Smith, Ole Wiskow, Kasper Roet, Xuan Huang, Kevin Eggan, Anne E. Carpenter, Robin J. Kleiman, Thomas L. Schwarz   +15 more
doaj   +1 more source

Development of Cordyceps javanica BE01 with enhanced virulence against Hyphantria cunea using polyethylene glycol-mediated protoplast transformation

Frontiers in Microbiology, 2022
Cordyceps javanica has promising application prospects as an entomopathogenic fungus with a wide range of hosts. To enhance the virulence of C. javanica, a polyethylene glycol (PEG)-mediated protoplast genetic transformation system was constructed ...
Wenxiu Wang, Yahong Wang, Guangping Dong, Fengmao Chen   +3 more
doaj   +1 more source

Generation of pathogenic TPP1 mutations in human stem cells as a model for neuronal ceroid lipofuscinosis type 2 disease

Stem Cell Research, 2021
Neuronal ceroid lipofuscinosis type 2 (CLN2 disease) is an autosomal recessive neurodegenerative disorder generally with onset at 2 to 4 years of age and characterized by seizures, loss of vision, progressive motor and mental decline, and premature death.
Li Ma, Adriana M. Prada, Michael Schmidt, Eric M. Morrow   +3 more
doaj   +1 more source

A zebrafish model of CLN2 disease is deficient in tripeptidyl peptidase 1 and displays progressive neurodegeneration accompanied by a reduction in proliferation [PDF]

Brain, 2013
Tripeptidyl peptidase 1 (TPP1) deficiency causes CLN2 disease, late infantile (or classic late infantile neuronal ceroid lipofuscinosis), a paediatric neurodegenerative disease of autosomal recessive inheritance. Patients suffer from blindness, ataxia, epilepsy and cognitive defects, with MRI indicating widespread brain atrophy, and profound neuron ...
Fahad, Mahmood, Sonia, Fu, Jennifer, Cooke, Stephen W, Wilson, Jonathan D, Cooper, Claire, Russell   +5 more
openaire   +2 more sources

Partial genetic suppression of a loss-of-function mutant of the neuronal ceroid lipofuscinosis-associated protease TPP1 in Dictyostelium discoideum

Disease Models & Mechanisms, 2015
Neuronal ceroid lipofuscinosis (NCL) is the most common childhood-onset neurodegenerative disease. NCL is inevitably fatal, and there is currently no treatment available.
Jonathan E. Phillips, Richard H. Gomer
doaj   +1 more source

Identification of three predictors of gastric cancer progression and prognosis

FEBS Open Bio, 2020
Abnormal gene expression is an established cause of gastric cancer (GC) initiation and progression. In this study, we aimed to identify several key genes that could be used to effectively predict progression and prognosis in patients with GC.
Kai Huang, Shuhua Chen, Rongzhang Xie, Pengpeng Jiang, Changjun Yu, Jinmei Fang, Xingcun Liu, Fazhi Yu   +7 more
doaj   +1 more source