Aspartoacylase gene knockout in the mouse: Impact on reproduction

Sankar Surendran, Sylvia Szucs, Stephen K. Tyring, Reuben Matalon

Research output: Contribution to journalArticlepeer-review

8 Scopus citations


Canavan disease (CD) is an autosomal recessive disorder caused by aspartoacylase (ASPA) gene mutations resulting enzyme deficiency. The homozygous knockout mouse for CD showed symptoms similar observed in patients with CD. Canavan disease leads to early death. Therefore, a role of ASPA in reproduction was investigated using the mouse model for CD. Homozygous (KO/KO) pups, produced by mating female heterozygous (KO/+) mouse with KO/+ males had approximately 12% death incidence rates in the first 2 months of life. KO/KO mothers mated with KO/+ males showed fetal death. KO/KO mothers produced fewer offspring compared to KO/+ mothers. These data suggest that ASPA is necessary for normal reproduction and postnatal survival.

Original languageEnglish (US)
Pages (from-to)281-283
Number of pages3
JournalReproductive Toxicology
Issue number2
StatePublished - 2005
Externally publishedYes


  • Aspartoacylase
  • Canavan disease
  • Fetal death
  • Knockout mouse
  • Postnatal death

ASJC Scopus subject areas

  • Toxicology


Dive into the research topics of 'Aspartoacylase gene knockout in the mouse: Impact on reproduction'. Together they form a unique fingerprint.

Cite this