rimeporide rimeporide rimeporide is an experimental drug for the treatment of duchenne muscular dystrophy, being developed by the esperare foundation. it has been granted orphan drug status by the european medicines agency. rimeporideclinical dataatc codenonelegal statuslegal status experimental identifiers iupac name n-(2-methyl-4,5-bis(methylsulfonyl)benzoyl)guanidine cas number187870-78-6pubchem cid9799487chemspider7975252uniiqh6b4v5743chemblchembl2107802chemical and physical dataformulac11h15n3o5s2molar mass333.38 g·mol−13d model (jsmol)interactive image smiles cc1cc(c(cc1c(=o)nc(=n)n)s(=o)(=o)c)s(=o)(=o)c inchi inchi=1s/c11h15n3o5s2/c1-6-4-8(20(2,16)17)9(21(3,18)19)5-7(6)10(15)14-11(12)13/h4-5h,1-3h3,(h4,12,13,14,15)key:gromeqpxdkrrie-uhfffaoysa-n mechanism of action the substance blocks an ion pump called sodium–hydrogen antiporter 1. while the exact mechanism is unknown, it is speculated that inhibition of this pump reduces ph, sodium and calcium overload in cells of patients with duchenne muscular dystrophy. history rimeporide was designed as a treatment for chronic heart failure. it was tested in seven phase i studies clinical trials in patients with congestive heart failure and some degree of renal insufficiency. subsequently, the drug was licensed to esperare, a swiss nonprofit organisation that aims at repositioning drugs for rare diseases. as of may 2015, the substance has demonstrated efficacy in several animal models of duchenne muscular dystrophy. it has also been recently tested in young boys with duchenne muscular dystrophy aged 6 to 11 years.