Table of Contents
Journal of Amino Acids
Volume 2014, Article ID 346809, 12 pages
Research Article

Development of a Novel Cysteine Sulfinic Acid Decarboxylase Knockout Mouse: Dietary Taurine Reduces Neonatal Mortality

1Laboratory of Cellular Immunology, Department of Developmental Neurobiology, New York State Institute for Basic Research in Developmental Disabilities, Staten Island, NY 10314, USA
2College of Veterinary Medicine, Konkuk University, Seoul 143-701, Republic of Korea
3Laboratory of Molecular Genetics, Department of Human Genetics, New York State Institute for Basic Research in Developmental Disabilities, Staten Island, NY 10314, USA

Received 16 October 2013; Revised 13 December 2013; Accepted 15 December 2013; Published 3 February 2014

Academic Editor: Guoyao Wu

Copyright © 2014 Eunkyue Park et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


We engineered a CSAD KO mouse to investigate the physiological roles of taurine. The disruption of the CSAD gene was verified by Southern, Northern, and Western blotting. HPLC indicated an 83% decrease of taurine concentration in the plasma of CSAD . Although CSAD generation (G)1 and G2 survived, offspring from G2 CSAD had low brain and liver taurine concentrations and most died within 24 hrs of birth. Taurine concentrations in G3 CSAD born from G2 CSAD treated with taurine in the drinking water were restored and survival rates of G3 CSAD increased from 15% to 92%. The mRNA expression of CDO, ADO, and TauT was not different in CSAD compared to WT and CSAD mRNA was not expressed in CSAD . Expression of Gpx 1 and 3 was increased significantly in CSAD and restored to normal levels with taurine supplementation. Lactoferrin and the prolactin receptor were significantly decreased in CSAD . The prolactin receptor was restored with taurine supplementation. These data indicated that CSAD KO is a good model for studying the effects of taurine deficiency and its treatment with taurine supplementation.