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

Brain gene expression profiles of Cln1 and Cln5 deficient mice unravels common molecular pathways underlying neuronal degeneration in NCL diseases.

BMC genomics - 28 Mar 2008

von Schantz Carina, Saharinen Juha, Kopra Outi, Cooper Jonathan D, Gentile Massimiliano, Hovatta Iiris, Peltonen Leena, Jalanko Anu

Abstract excerpt

BACKGROUND: The neuronal ceroid lipofuscinoses (NCL) are a group of children's inherited neurodegenerative disorders, characterized by blindness, early dementia and pronounced cortical atrophy. The similar pathological and clinical profiles of the different forms of NCL suggest that common disease mechanisms may be involved. To explore the NCL-associated disease pathology and molecular pathways, we have...

Read the complete abstract on PubMed

Topics

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

Share to Growth Cones
Share to Immediate-Early Proteins

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Share to Lysosomal Membrane Proteins

Share to Neuronal Ceroid-Lipofuscinoses

Share to rab3 GTP-Binding Proteins

Share to Receptor-Like Protein Tyrosine Phosphatases, Class 2

Share to Synapsins

Share to Thiolester Hydrolases

Source

Open in PubMed Open full text

Identifiers

PMID: 18371231
DOI: 10.1186/1471-2164-9-146
PMCID: PMC2323392

Genes

NCL
CLN5

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

Brain gene expression profiles of Cln1 and Cln5 deficient mice unravels common molecular pathways underlying neuronal degeneration in NCL diseases.PMID 18371231Read ↗

Exacerbated neuronal ceroid lipofuscinosis phenotype in Cln1/5 double-knockout mice.Semantic proximity Read ↗

A mouse model for Finnish variant late infantile neuronal ceroid lipofuscinosis, CLN5, reveals neuropathology associated with early agingSemantic proximity Read ↗

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

Retinal Degeneration In A Mouse Model Of CLN5 Disease Is Associated With Compromised AutophagySemantic proximity Read ↗

The mouse ortholog of the neuronal ceroid lipofuscinosis CLN5 gene encodes a soluble lysosomal glycoprotein expressed in the developing brainSemantic proximity Read ↗

Analysis of Potential Biomarkers and Modifier Genes Affecting the Clinical Course of CLN3 DiseaseSemantic proximity Read ↗

Loss of Cln5 leads to altered Gad1 expression and deficits in interneuron development in miceSemantic proximity Read ↗

Progressive thalamocortical neuron loss in Cln5 deficient mice: Distinct effects in Finnish variant late infantile NCL.Semantic proximity Read ↗

CLN5 mutations are frequent in juvenile and late-onset non-Finnish patients with NCL.Semantic proximity Read ↗

Proteomic and functional analyses in disease models reveal CLN5 protein involvement in mitochondrial dysfunctionSemantic proximity Read ↗

Recent insights into the networking of CLN genes and proteins in mammalian cells.Semantic proximity Read ↗

Moving towards a new era of genomics in the neuronal ceroid lipofuscinoses.Semantic proximity Read ↗

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