Aberrant upregulation of the glycolytic enzyme PFKFB3 in CLN7 neuronal ceroid lipofuscinosis
CLN7 neuronal ceroid lipofuscinosis is an inherited lysosomal storage disease typically with childhood onset of neurodegenerative symptoms. Here the authors report that in a mouse model of CLN7 disease neuronal reactive oxygen species and the activity of glycolytic enzyme PFKFB3 are increased, while...
Main Authors: | Irene Lopez-Fabuel, Marina Garcia-Macia, Costantina Buondelmonte, Olga Burmistrova, Nicolo Bonora, Paula Alonso-Batan, Brenda Morant-Ferrando, Carlos Vicente-Gutierrez, Daniel Jimenez-Blasco, Ruben Quintana-Cabrera, Emilio Fernandez, Jordi Llop, Pedro Ramos-Cabrer, Aseel Sharaireh, Marta Guevara-Ferrer, Lorna Fitzpatrick, Christopher D. Thompton, Tristan R. McKay, Stephan Storch, Diego L. Medina, Sara E. Mole, Peter O. Fedichev, Angeles Almeida, Juan P. Bolaños |
---|---|
Format: | Article |
Language: | English |
Published: |
Nature Portfolio
2022-01-01
|
Series: | Nature Communications |
Online Access: | https://doi.org/10.1038/s41467-022-28191-1 |
Similar Items
-
An Unusual Presentation of Neuronal Ceroid Lipofuscinosis With CLN6 Mutation
by: Shahin Koohmanaee, et al.
Published: (2023-01-01) -
in vivo localization of the neuronal ceroid lipofuscinosis proteins, CLN3 and CLN7, at endogenous expression levels
by: Alamin Mohammed, et al.
Published: (2017-07-01) -
Etiology of anxious and fearful behavior in juvenile neuronal ceroid lipofuscinosis (CLN3 disease)
by: John R. Ostergaard
Published: (2023-04-01) -
Exacerbated neuronal ceroid lipofuscinosis phenotype in Cln1/5 double-knockout mice
by: Tea Blom, et al.
Published: (2013-03-01) -
Novel interactions of CLN5 support molecular networking between Neuronal Ceroid Lipofuscinosis proteins
by: Jalanko Anu, et al.
Published: (2009-11-01)