Discover Community Create You

Loading publication...

Noodle always counts identifier-free feature events for service operation. With your permission, Google Analytics can also estimate audience and engagement. Search text, publication identifiers, cookies, IP addresses and browser fingerprints are never included in Noodle's first-party event counters.

Privacy details

Article

Batten disease (JNCL) is linked to disturbances in mitochondrial, cytoskeletal, and synaptic compartments.

Journal of neuroscience research - 1 Oct 2006

Luiro Kaisu, Kopra Outi, Blom Tomas, Gentile Massimiliano, Mitchison Hannah M, Hovatta Iiris, Törnquist Kid, Jalanko Anu

Abstract excerpt

Intracellular pathways leading to neuronal degeneration are poorly understood in the juvenile neuronal ceroid lipofuscinosis (JNCL, Batten disease), caused by mutations in the CLN3 gene. To elucidate the early pathology, we carried out comparative global transcript profiling of the embryonic, primary cultures of the Cln3-/- mouse neurons. Statistical and functional analyses delineated three major cellular...

Read the complete abstract on PubMed

Topics

Share this publication in a Topic to start or enrich a Post.

Share to Cytoskeleton
Share to Dynactin Complex

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Share to Embryo, Mammalian

Share to Microscopy, Electron, Transmission

Share to Microtubule-Associated Proteins

Share to Molecular Chaperones

Share to Neuronal Ceroid-Lipofuscinoses

Share to Synapses

Source

Identifiers

PMID: 16941499
DOI: 10.1002/jnr.21015

Genes

CLN3

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

Related research

Semantic proximity does not establish scientific evidence.

Research trail→

Click a neighbor to travelStep 1 · 12 closest

Batten disease (JNCL) is linked to disturbances in mitochondrial, cytoskeletal, and synaptic compartments.PMID 16941499Read ↗

Membrane trafficking and mitochondrial abnormalities precede subunit c deposition in a cerebellar cell model of juvenile neuronal ceroid lipofuscinosisSemantic proximity Read ↗

Current Insights in Elucidation of Possible Molecular Mechanisms of the Juvenile Form of Batten Disease.Semantic proximity Read ↗

Targeted disruption of the Cln3 gene provides a mouse model for Batten disease. The Batten Mouse Model Consortium [corrected].Semantic proximity Read ↗

Loss of CLN3, the gene mutated in juvenile neuronal ceroid lipofuscinosis, leads to metabolic impairment and autophagy induction in retinal pigment epithelium.Semantic proximity Read ↗

The Batten disease gene CLN3 is required for the response to oxidative stress.Semantic proximity Read ↗

Thalamocortical neuron loss and localized astrocytosis in the Cln3Deltaex7/8 knock-in mouse model of Batten disease.Semantic proximity Read ↗

Defective intracellular transport of CLN3 is the molecular basis of Batten disease (JNCL)Semantic proximity Read ↗

Molecular correlates of axonal and synaptic pathology in mouse models of Batten diseaseSemantic proximity Read ↗

Large-scale phenotyping of an accurate genetic mouse model of JNCL identifies novel early pathology outside the central nervous system.Semantic proximity Read ↗

Biosynthesis and Intracellular Targeting of the CLN3 Protein Defective in Batten DiseaseSemantic proximity Read ↗

Defective synaptic transmission causes disease signs in a mouse model of juvenile neuronal ceroid lipofuscinosisSemantic proximity Read ↗

Synapse alterations precede neuronal damage and storage pathology in a human cerebral organoid model of CLN3-juvenile neuronal ceroid lipofuscinosis.Semantic proximity Read ↗

⌁Select a neighboring publication to make it the new centre.