Otoprotective Effects Of Pyroglutamate Helix B Surface Peptide In Cdh23^erl/erl Mutant Mice With Early-Onset Hearing Loss

The Cdh23^erl/erl mice?erl mice?was proven to be a novel mouse model of human autosomal recessive nonsyndromic deafness?DFNB12?.Our previous study has shown that apoptosis is one of the most important pathological mechanisms leading to hearing loss and hair cell death in Cdh23erl/erl mice.Pyroglutamate helix B surface peptide?pHBSP;ARA-290?is a peptide newly derived from the tertiary structure of EPO,which could selectively activate ? common receptor??CR;CD131?subunits without interacting with EPO receptor?EPOR?homodimer.? common receptor was upregulated in Cdh23erl/erl mice detected by immunofluorescence and real time quantitative PCR.In addition,we investigated its otoprotective effects in Cdh23erl/erl mice.Our results showed that pHBSP significantly improved the hearing level and reduced hair cell death percentage of Cdh23erl/erl mice compared with the PBS-treated Cdh23erl/erl mice and/or untreated Cdh23erl/erl mice.The data suggest that pHBSP could be a promising new drug in the prevention of human DFNB12.