Aspartoacylase-LacZ Knockin Mice: An Engineered Model of Canavan Disease

  title={Aspartoacylase-LacZ Knockin Mice: An Engineered Model of Canavan Disease},
  author={Nadine Mersmann and Dmitri Tkachev and Ruth Jelinek and Philipp Thomas R{\"o}th and Wiebke M{\"o}bius and Torben Ruhwedel and Sabine R{\"u}hle and Wolfgang Weber-Fahr and Alexander Sartorius and Matthias Klugmann},
  journal={PLoS ONE},
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) gene under the control of the aspa regulatory elements. X-Gal staining in known ASPA… 

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