Results 51 to 60 of about 87,249 (163)

Effect of collection, transport, processing and storage of blood specimens on the activity of lysosomal enzymes in plasma and leukocytes

Brazilian Journal of Medical and Biological Research, 2000
This study was designed to evaluate the effect of different conditions of collection, transport and storage on the quality of blood samples from normal individuals in terms of the activity of the enzymes ß-glucuronidase, total hexosaminidase ...
M. Burin, C. Dutra-Filho, J. Brum, T. Mauricio, M. Amorim, R. Giugliani   +5 more
doaj   +1 more source

Impaired β-glucocerebrosidase activity and processing in frontotemporal dementia due to progranulin mutations

Acta Neuropathologica Communications, 2019
Loss-of-function mutations in progranulin (GRN) are a major autosomal dominant cause of frontotemporal dementia. Most pathogenic GRN mutations result in progranulin haploinsufficiency, which is thought to cause frontotemporal dementia in GRN mutation ...
Andrew E. Arrant, Jonathan R. Roth, Nicholas R. Boyle, Shreya N. Kashyap, Madelyn Q. Hoffmann, Charles F. Murchison, Eliana Marisa Ramos, Alissa L. Nana, Salvatore Spina, Lea T. Grinberg, Bruce L. Miller, William W. Seeley, Erik D. Roberson   +12 more
doaj   +1 more source

HIV Tat Domain Improves Cross-correction of Human Galactocerebrosidase in a Gene- and Flanking Sequence-dependent Manner

Molecular Therapy: Nucleic Acids, 2013
Krabbe disease is a devastating neurodegenerative lysosomal storage disorder caused by a deficiency of β-galactocerebrosidase (GALC). Gene therapy is a promising therapeutic approach for Krabbe disease.
Xing-Li Meng, Yoshikatsu Eto, Raphael Schiffmann, Jin-Song Shen   +3 more
doaj   +1 more source

Evidence of Lysosomal β-Hexosaminidase Enzymatic Activity Associated with Extracellular Vesicles: Potential Applications for the Correction of Sandhoff Disease

Journal of Functional Biomaterials
Extracellular vesicles (EVs) can be isolated from biological fluids and cell culture medium. Their nanometric dimension, relative stability, and biocompatibility have raised considerable interest for their therapeutic use as delivery vehicles of ...
Eleonora Calzoni, Giada Cerrotti, Krizia Sagini, Federica Delo, Sandra Buratta, Roberto Maria Pellegrino, Husam B. R. Alabed, Federica Fratini, Carla Emiliani, Lorena Urbanelli   +9 more
doaj   +1 more source

Proteolysis of Amyloid β by Lysosomal Enzymes as a Function of Fibril Morphology. [PDF]

ACS Omega, 2021
Lambeth TR, Julian RR.
europepmc   +1 more source

KCTD7 mutations impair the trafficking of lysosomal enzymes through CLN5 accumulation to cause neuronal ceroid lipofuscinoses. [PDF]

Sci Adv, 2022
Wang Y, Cao X, Liu P, Zeng W, Peng R, Shi Q, Feng K, Zhang P, Sun H, Wang C, Wang H.   +10 more
europepmc   +1 more source

Evaluation of artificial signal peptides for secretion of two lysosomal enzymes in CHO cells. [PDF]

Biochem J, 2021
Cheng KW, Wang F, Lopez GA, Singamsetty S, Wood J, Dickson PI, Chou TF.   +6 more
europepmc   +1 more source

A Novel Method of Imaging Lysosomes in Living Human Mammary Epithelial Cells

Molecular Imaging, 2003
Cancer cells invade by secreting degradative enzymes which, under normal conditions, are sequestered in lysosomal vesicles. The ability to noninvasively label lysosomes and track lysosomal trafficking would be extremely useful to understand the ...
Kristine Glunde, Sandra E. Guggino, Yoshitaka Ichikawa, Zaver M. Bhujwalla   +3 more
doaj   +1 more source

Diabetes mellitus alters lysosomal enzymes activity in rabbit epididymis

Current Issues in Pharmacy and Medical Sciences, 2009
Diabetes is a metabolic disease caused by inappropriate secretion and/or function of insulin, which may disturb morphology and function of various internal organs. The aim of the study was to evaluate the activity of selected lysosomal enzymes.
Zbigniew Wójtowicz, Wojciech Dworzański, Mariusz Klepacki, Teresa Hermanowicz-Dryka, Emilia Potembska, Jolanta Lisiecka, Tomasz Horoch, Jakub Dworzański, Agata Kędzierawska-Kurylcio, Monika Cendrowska-Pinkosz, Franciszek Burdan   +10 more
doaj  

Microglia degrade Alzheimer’s amyloid-beta deposits extracellularly via digestive exophagy

Cell Reports
Summary: How microglia digest Alzheimer’s fibrillar amyloid-beta (Aβ) plaques that are too large to be phagocytosed is not well understood. Here, we show that primary microglial cells create acidic extracellular compartments, lysosomal synapses, on model
Rudy G. Jacquet, Fernando González Ibáñez, Katherine Picard, Lucy Funes, Mohammadparsa Khakpour, Gunnar K. Gouras, Marie-Ève Tremblay, Frederick R. Maxfield, Santiago Solé-Domènech   +8 more
doaj   +1 more source