Phenotypic characterization of a mouse model of juvenile neuronal ceroid lipofuscinosis.

doi:10.1016/j.nbd.2007.08.017

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Article

Phenotypic characterization of a mouse model of juvenile neuronal ceroid lipofuscinosis.

Neurobiology of disease - 1 Feb 2008

Katz Martin L, Johnson Gary S, Tullis Gregory E, Lei Bo

Abstract excerpt

Juvenile neuronal ceroid lipofuscinosis (JNCL) is an autosomal recessively inherited neurodegenerative disorder that results from mutations in the CLN3 gene. JNCL is characterized by accumulation of autofluorescent lysosomal storage bodies, vision loss, seizures, progressive cognitive and motor decline, and premature death. Studies were undertaken to characterize the neuronal ceroid lipofuscinosis phenotype in a...

Topics

Age Factors
Animals
Behavior, Animal
Body Weight
Disease Models, Animal
Electroretinography
Exploratory Behavior
Learning
Longevity
Membrane Glycoproteins
Mice
Mice, Inbred C57BL
Mice, Knockout
Molecular Chaperones
Neuronal Ceroid-Lipofuscinoses

Source

Open in PubMed Open full text

Identifiers

PMID: 17962032
DOI: 10.1016/j.nbd.2007.08.017
PMCID: PMC2268761

Genes

CLN3

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 30 Aug 2026.

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Phenotype

Reflex, Pupillary

Retina