As we found that the expression levels of the two main H2S producing enzymes, cystathionine ¦Â synthase and cystathionine ¦Ã lyase, were lower in WS cells compared to normal, we investigated the effect of administration of H2S as NaHS (50 ¦ÌM). NaHS treatment blocked mTOR activity, abrogated protein aggregation and normalized the phenotype of WS cells. Similar results were obtained by treatment with the mTOR inhibitor rapamycin.
This is the first report suggesting that hydrogen sulfide administered as NaHS restores proteostasis and cellular morphological phenotype of WS cells and hints to the importance of transsulfuration pathway in WS.