A compound represented by the following formula (I) or a pharmaceutically acceptable oxide, salt or solvate thereof for use in the treatment of a protein-mediated disease containing valosin (VCP): ** Formula ** wherein: R is phenyl, pyridyl, quinolinyl or thiophenyl, each of which may have one or more Ra substituents； each Ra is independently selected from halo； I rent； substituted alkyl； alkenyl； substituted alkenyl； alkynyl； substituted alkynyl； aryl； substituted aryl； cycloalkyl； substituted cycloalkyl； cycloalkenyl； substituted cycloalkenyl； heteroaryl； substituted heteroaryl； heterocyclyl； substituted heterocyclyl； Not me； substituted amino represented by -NR21R22 in which R21 and R22 are independently selected from hydrogen, R3 and SO2-R30 or R21 and R22, together with the nitrogen to which they are attached, form a substituted heterocyclyl or heterocyclyl, provided that R21 and R22 do not are simultaneously hydrogen； cyano； nitro； acyl selected from HC (O) -, R30-C (O) - and NR27R28 -NR26-C (O) - in which R26, R27 and R28 are independently selected from hydrogen, R30, -C (O) O-R30 and SO2-R30 or R27 and R28, together with the nitrogen to which they are attached, form a substituted heterocyclyl or heterocyclyl； acylamino represented by -NR20C (O) -R30 in which R20 is hydrogen, alkyl or substituted alkyl； alkoxy； substituted alkoxy； carboxyl； carboxyl ester represented by -C (O) O-R30； thioacyl selected from H-C (S) - and R30-C (S) -； thiocyanate； thiol； alkylthio； substituted alkylthio； substituted sulfonyl represented by -SO2-R30； aminosulfonyl represented by -SO2NR23R24 wherein R23 and R24 are independently selected from hydrogen, R30, hydroxy, alkoxy, substituted alkoxy, amino, the above-defined substituted amino and acylamino defined above, or R23 and R24, together with the nitrogen to which are attached, form a heterocyclyl or substituted heterocyclyl； and aminocarbonyl represented by -C (O) NR23R24 in which R23 and R24 are defined as before； R30 is selec
