Results 81 to 90 of about 7,909 (155)

Progranulin is neurotrophic in vivo and protects against a mutant TDP-43 induced axonopathy.

PLoS ONE, 2010
Mislocalization, aberrant processing and aggregation of TAR DNA-binding protein 43 (TDP-43) is found in the neurons affected by two related diseases, amyotrophic lateral sclerosis (ALS) and frontotemporal lobe dementia (FTLD).
Angela S Laird, Annelies Van Hoecke, Louis De Muynck, Mieke Timmers, Ludo Van den Bosch, Philip Van Damme, Wim Robberecht   +6 more
doaj   +1 more source

Optimization of Progranulin Gene Therapy, a Potential Treatment for Frontotemporal Dementia [PDF]

, 2023
Heterozygous loss of function mutations in the progranulin gene cause frontotemporal dementia, characterized by deficits in social behavior, language comprehension and motor function.
Kashyap, Shreya Natesh
core  

Optimal plasma progranulin cutoff value for predicting null progranulin mutations in neurodegenerative diseases : a multicenter Italian study

, 2012
Background: Recently, attention was drawn to a role for progranulin in the central nervous system with the identification of mutations in the progranulin gene (GRN) as an important cause of frontotemporal lobar degeneration.
E. Piccoli, F. Tagliavini, E. Scarpini, E. Stoppani, C. Fenoglio, V. Albertini, G. Binetti, L. F. Agnati, R. Ghidoni, A. Paterlini, E. Pegoiani, G. Rossi, L. Benussi, D. Galimberti, M. Glionna, M. Morbin   +15 more
core   +2 more sources

Progranulin as a biomarker and potential therapeutic agent [PDF]

, 2019
Progranulin is a cysteine-rich secreted protein with diverse pleiotropic actions and participates in several processes, such as inflammation or tumorigenesis.
Scotece, M, Conde, J, Gonzalez-Gay, MA, Gualillo, O, Abella, V, Mera, A, Lago, F, Pino, J, Gómez, R, Mobasheri, Ali   +9 more
core   +1 more source

Genetic Regulation of Neuronal Progranulin Reveals a Critical Role for the Autophagy-Lysosome Pathway [PDF]

, 2019
Deficient progranulin levels cause dose-dependent neurological syndromes: haploinsufficiency leads to frontotemporal lobar degeneration (FTLD) and nullizygosity produces adult-onset neuronal ceroid lipofuscinosis.
Mason, Amanda R, Finkbeiner, Steven, Elia, Lisa P, Alijagic, Amela   +3 more
core   +1 more source

Reduction of Sphingomyelinases Associated with Progranulin Deficiency and Frontotemporal Dementia [PDF]

, 2023
Frontotemporal dementia (FTD) is a leading cause of early-onset dementia and has a significant socioeconomic burden due to difficulties in diagnosis and delay to diagnosis. FTD is a clinically, pathologically, and genetically heterogeneous disorder.
Boyle, Nicholas R
core  

Progranulin Biology: Small Molecule Enhancers of Progranulin Expression and Biochemical Analysis of Granulin Receptors

, 2012
Frontotemporal dementia (FTD) is the second most common presenile dementia syndrome. Mutations in the GRN gene account for about 20% of patients with familial FTD.
Cenik, Basar 1981-
core   +1 more source

Supplementary Material for: Cerebrospinal Fluid Progranulin, but Not Serum Progranulin, Is Reduced in GRN-Negative Frontotemporal Dementia

, 2016
Background and Objective: Reduced progranulin levels are a hallmark of frontotemporal dementia (FTD) caused by loss-of-function (LoF) mutations in the progranulin gene (GRN).
Deuschle C. (3226044), Blauwendraat C. (3226038), Maetzler W. (3226059), Wilke C. (3226062), Metzger F.G. (3226041), Jansen I.E. (3226047), Synofzik M. (3226056), Hobert M.A. (3226065), Gillardon F. (3226053), Gasser T. (3226068), Heutink P. (3226050)   +10 more
core   +1 more source

Benzoxazole-derivatives enhance progranulin expression and reverse the aberrant lysosomal proteome caused by GRN haploinsufficiency

Nature Communications
Heterozygous loss-of-function mutations in the GRN gene are a major cause of hereditary frontotemporal dementia. The mechanisms linking frontotemporal dementia pathogenesis to progranulin deficiency are not well understood, and there is currently no ...
Rachel Tesla, Charlotte Guhl, Gordon C. Werthmann, Danielle Dixon, Basar Cenik, Yesu Addepalli, Jue Liang, Daniel M. Fass, Zachary Rosenthal, Stephen J. Haggarty, Noelle S. Williams, Bruce A. Posner, Joseph M. Ready, Joachim Herz   +13 more
doaj   +1 more source

Evaluation of progranulin and inflammatory markers in the diagnosis of acute cholecystitis

Medicine Science
This study aimed to investigate the role of inflammatory markers and progranulin levels in diagnosing cholecystitis, assessing their potential as diagnostic biomarkers. The research involved 54 patients diagnosed with acute cholecystitis in the emergency
Ali Ihsan Kilci, Muhammed Seyithanoglu, Erhan Kaya, Hakan Hakkoymaz, Muhammed Semih Gedik, Omer Faruk Kucuk   +5 more
doaj   +1 more source