Back to search

Article

Reversible synaptic deficits in early-stage Batten disease

2025-11-12

Abstract excerpt

Juvenile neuronal ceroid lipofuscinosis (JNCL, Batten Disease) is a childhood-onset, neurodegenerative, lysosomal storage disorder caused by mutations in the lysosomal gene CLN3 . Progressive cognitive decline is a key clinical manifestation of JNCL, and no definitive treatment is currently available. The precise function of CLN3 in neurons remains unclear, hindering the development of targeted therapies. Using p...

Topics

Open a Topic to create a Post that cites this publication.

Identifiers and source

Literature Corpus work
7b987c36-7741-5bce-ab28-2db2a75279e6
DOI
10.1101/2025.11.12.687979
Open publication

Related research

Semantic proximity does not establish scientific evidence.

Click a neighbor to travelStep 1 · 12 closest
Interactive article relationship graphSelect a related publication card to move it into the centre and load its closest explainable connections. Solid lines are source-backed structured connections. Dashed lines are semantic discovery signals and are not scientific evidence.
Reversible synaptic deficits in early-stage Batten diseaseDOI 10.1101/2025.11.12.687979
Defective synaptic transmission causes disease signs in a mouse model of juvenile neuronal ceroid lipofuscinosisSemantic proximity
Neuronal genetic rescue normalizes brain network dynamics in a lysosomal storage disorder despite persistent storage accumulationSemantic proximity
Current Insights in Elucidation of Possible Molecular Mechanisms of the Juvenile Form of Batten Disease.Semantic proximity
Membrane trafficking and mitochondrial abnormalities precede subunit c deposition in a cerebellar cell model of juvenile neuronal ceroid lipofuscinosisSemantic proximity
Batten disease (JNCL) is linked to disturbances in mitochondrial, cytoskeletal, and synaptic compartments.Semantic proximity
Defective intracellular transport of CLN3 is the molecular basis of Batten disease (JNCL)Semantic proximity
A Knock-In Reporter Model of Batten DiseaseSemantic proximity
A novel porcine model of CLN3 Batten disease recapitulates clinical phenotypesSemantic proximity
A novel porcine model of CLN3 Batten disease recapitulates clinical phenotypes.Semantic proximity
Revisiting the neuronal localization and trafficking of CLN3 in juvenile neuronal ceroid lipofuscinosis.Semantic proximity
Synapse alterations precede neuronal damage and storage pathology in a human cerebral organoid model of CLN3-juvenile neuronal ceroid lipofuscinosis.Semantic proximity
Neurodegeneration and Epilepsy in a Zebrafish Model of CLN3 Disease (Batten Disease)Semantic proximity
Select a neighboring publication to make it the new centre.