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Article

The neuronal ceroid-lipofuscinoses.

Developmental disabilities research reviews - 1 Jan 2013

Bennett Michael J, Rakheja Dinesh

Abstract excerpt

The neuronal ceroid-lipofuscinoses (NCL's, Batten disease) represent a group of severe neurodegenerative diseases, which mostly present in childhood. The phenotypes are similar and include visual loss, seizures, loss of motor and cognitive function, and early death. At autopsy, there is massive neuronal loss with characteristic storage in remaining neurons. Neurons appear to die because of increased rates of...

Topics

Aminopeptidases
Animals
Dipeptidyl-Peptidases and Tripeptidyl-Peptidases
Humans
Neuronal Ceroid-Lipofuscinoses
Phenotype
Serine Proteases
Thiolester Hydrolases
Tripeptidyl-Peptidase 1

Source

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Identifiers

PMID: 23798013
DOI: 10.1002/ddrr.1118

Genes

NCL
CLN3
TPP1

Provenance

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

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