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Article

Aspartoacylase-lacZ knockin mice: an engineered model of Canavan disease.

PloS one - 1 Jan 2011

Mersmann Nadine, Tkachev Dmitri, Jelinek Ruth, Röth Philipp Thomas, Möbius Wiebke, Ruhwedel Torben, Rühle Sabine, Weber-Fahr Wolfgang, Sartorius Alexander, Klugmann Matthias

Abstract excerpt

Canavan Disease (CD) is a recessive leukodystrophy caused by loss of function mutations in the gene encoding aspartoacylase (ASPA), an oligodendrocyte-enriched enzyme that hydrolyses N-acetylaspartate (NAA) to acetate and aspartate. The neurological phenotypes of different rodent models of CD vary considerably. Here we report on a novel targeted aspa mouse mutant expressing the bacterial β-Galactosidase (lacZ)...

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Identifiers

PMID: 21625469
DOI: 10.1371/journal.pone.0020336
PMCID: PMC3098885

Genes

ASPA

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 6 Sept 2026. Manifest publication-graph-cohort-2026-09-06.1.

Source receipt: pubmed, retrieved 6 Sept 2026.

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