Results 1 to 10 of about 87,336 (259)

GRASP55 maintains lysosome function by controlling sorting of lysosomal enzymes at the Golgi [PDF]

EMBO Reports
Lysosomes are multifunctional organelles that play important roles in cellular recycling, signaling, and homeostasis, relying on precise trafficking and activation of lysosomal enzymes.
Julian Nüchel, Maryam Omidi, Stephanie A Fernandes, Marina Tauber, Sandra Pohl, Markus Plomann, Constantinos Demetriades   +6 more
doaj   +2 more sources

Engineering of GlcNAc-1-Phosphotransferase for Production of Highly Phosphorylated Lysosomal Enzymes for Enzyme Replacement Therapy [PDF]

Molecular Therapy: Methods & Clinical Development, 2017
Several lysosomal enzymes currently used for enzyme replacement therapy in patients with lysosomal storage diseases contain very low levels of mannose 6-phosphate, limiting their uptake via mannose 6-phosphate receptors on the surface of the deficient ...
Lin Liu, Wang-Sik Lee, Balraj Doray, Stuart Kornfeld   +3 more
doaj   +2 more sources

Progranulin acts as a shared chaperone and regulates multiple lysosomal enzymes [PDF]

Genes and Diseases, 2017
Multifunctional factor progranulin (PGRN) plays an important role in lysosomes, and its mutations and insufficiency are associated with lysosomal storage diseases, including neuronal ceroid lipofuscinosis and Gaucher disease (GD).
Jinlong Jian, Aubryanna Hettinghouse, Chuan-ju Liu   +2 more
doaj   +2 more sources

Lysosomal enzymes and mannose 6-phosphate receptors in the lacrimal drainage system: Evidence and its potential implications

Indian Journal of Ophthalmology, 2018
Purpose: To investigate the presence and patterns of lysosomal enzymes and mannose 6-phosophate receptor (MPRs) in human lacrimal drainage system. Methods: The study was performed on healthy lacrimal sacs and nasolacrimal ducts obtained from exenteration
Mohammad Javed Ali, Ashapogu Venugopal, Kavyashree Sakharayapatna Ranganath, Nadimpalli Siva Kumar   +3 more
doaj   +2 more sources

Biomarkers of oxidative stress, biochemical changes, and the activity of lysosomal enzymes in the livers of rainbow trout (Oncorhynchus mykiss Walbaum) vaccinated against yersiniosis before a Yersinia ruckeri challenge [PDF]

Journal of Veterinary Research
This study aimed to evaluate biomarkers of oxidative stress (2-thiobarbituric acid reactive substances, aldehyde and ketone derivatives of oxidatively modified proteins and total antioxidant capacity), the activity of antioxidant enzymes (superoxide ...
Kurhaluk Natalia, Grudniewska Joanna, Pękala-Safińska Agnieszka, Pajdak-Czaus Joanna, Terech-Majewska Elżbieta, Platt-Samoraj Aleksandra, Tkaczenko Halina   +6 more
doaj   +2 more sources

Targeted delivery of glucocerebrosidase to lysosomes: The LYSOTAC (LYSOsome-TArgeting Chimera) technology [PDF]

Asian Journal of Pharmaceutical Sciences
Lysosomal storage diseases (LSDs) are a group of inherited metabolic disorders caused by misfolding of lysosomal proteins and their degradation via endoplasmic reticulum-associated degradation (ERAD).
Hee-Yeon Kim, Eun Nam Choi, Gee Eun Lee, Sanghwa Yoon, Su Ran Mun, Eui Jung Jung, Minji Kim, Hyomin Lim, Yang Jae Kang, Woo-Jae Park, Yong Tae Kwon, Joo-Won Park   +11 more
doaj   +2 more sources

Lysosomal storage diseases [PDF]

Journal of Veterinary and Animal Sciences, 2021
Lysosomes play a pivotal role in cellular processes through an active interplay of enzymes, lysosomal membrane proteins, and cytosolic proteins. Lysosomal storage diseases are a group of inherited and acquired disorders.
Smitha Rose Georgy
doaj   +1 more source

Loss of the batten disease protein CLN3 leads to mis-trafficking of M6PR and defective autophagic-lysosomal reformation

Nature Communications, 2023
Batten disease, one of the most devastating types of neurodegenerative lysosomal storage disorders, is caused by mutations in CLN3. Here, we show that CLN3 is a vesicular trafficking hub connecting the Golgi and lysosome compartments.
Alessia Calcagni’, Leopoldo Staiano, Nicolina Zampelli, Nadia Minopoli, Niculin J. Herz, Giuseppe Di Tullio, Tuong Huynh, Jlenia Monfregola, Alessandra Esposito, Carmine Cirillo, Aleksandar Bajic, Mahla Zahabiyon, Rachel Curnock, Elena Polishchuk, Luke Parkitny, Diego Luis Medina, Nunzia Pastore, Peter J. Cullen, Giancarlo Parenti, Maria Antonietta De Matteis, Paolo Grumati, Andrea Ballabio   +21 more
doaj   +1 more source

Mannose-6-phosphate glycan for lysosomal targeting: various applications from enzyme replacement therapy to lysosome-targeting chimeras

Animal Cells and Systems, 2022
A lysosome, an acidic membrane-bound organelle, contains hydrolytic enzymes to digest macromolecules for recycling. Many lysosomal enzymes (LEs) traffic to the lysosome through the mannose-6-phosphate (M6P)-dependent pathway.
Jinho Seo, Doo-Byoung Oh
doaj   +1 more source

Supplementation with β-carotene aids minimize inflammation in monosodium urate crystal- induced gouty arthritis in Wistar albino rats

Brazilian Journal of Pharmaceutical Sciences, 2022
Gout is a form of metabolic arthritis originated on grounds of increased accumulation of monosodium urate (MSU) crystals in joints. Current study focuses on anti-arthritic activities of β-carotene on MSU crystal-induced gouty arthritis rats in comparison
Lavinya B Udhaya, Aline Ubrewe Rutera, K Ram Kumar, N Sangeetha, P Manisha, Sabina Evan Prince   +5 more
doaj   +1 more source