Rimeporide

Rimeporide

Clinical data
ATC code	none
Legal status
Legal status	Experimental
Identifiers
IUPAC name N-(2-methyl-4,5-bis(methylsulfonyl)benzoyl)guanidine
CAS Number	187870-78-6
PubChem CID	9799487
ChemSpider	7975252
UNII	QH6B4V5743
ChEMBL	ChEMBL2107802
Chemical and physical data
Formula	C11H15N3O5S2
Molar mass	333.38 g·mol−1
3D 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

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.

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.

See also

Other drugs for Duchenne muscular dystrophy

• Ataluren
• Biostrophin (experimental)
• Idebenone (experimental)