Abstract: A compound represented by the following formula (1) is provided: wherein X represents a sulfur atom or —CH?CH—; A1 to A4 each independently represent a carbon atom or a nitrogen atom, and at least one of A1 to A4 is a nitrogen atom; R1 represents any one of a 1,2,3,4-tetrahydroquinolyl group (or a 3,4-dihydro-1(2H)-quinolyl group), a 3,4-dihydro-4,4-dimethyl-1(2H)-quinolyl group, a 2,3,4,5-tetrahydro-1H-1-benzazepinyl group, or a substituent represented by the following formula (2): wherein R4 represents a 2,3,4-trifluorophenyl group, a 4-iodophenyl group, a 2,3-difluorophenyl group, a 3,5-difluorophenyl group, a 5-fluoro-2-methylphenyl group, a 3-pentafluorosulfanylphenyl group, a 2,6-dimethylphenyl group, a 4-benzyloxyphenyl group, a 3,5-bis(trifluoromethyl)biphenyl group, a 4-tert-butylphenyl group, a 3-methoxyphenyl group, an unsubstituted or substituted pyridyl group, or an unsubstituted or substituted naphthyl group; R5 represents a hydrogen atom, a lower alkyl group, a lower alkenyl group, a l

Abstract: It is an object of the present invention to provide a compound inhibiting the binding between ALS-related mutant SOD1 and Derlin-1, a medicament comprising the compound, and a method for treating ALS by administering the medicament to a patient.