[HTML][HTML] Spermine synthase deficiency leads to deafness and a profound sensitivity to α-difluoromethylornithine*♦

X Wang, S Levic, MA Gratton, KJ Doyle… - Journal of biological …, 2009 - ASBMB
Male gyro (Gy) mice, which have an X chromosomal deletion inactivating the SpmS and
Phex genes, were found to be profoundly hearing impaired. This defect was due to alteration
in polyamine content due to the absence of spermine synthase, the product of the SpmS
gene. It was reversed by breeding the Gy strain with CAG/SpmS mice, a transgenic line that
ubiquitously expresses spermine synthase under the control of a composite cytomegalovirus-
IE enhancer/chicken β-actin promoter. There was an almost complete loss of the …