Abstract: A modified oligonucleotide having an activity of inhibiting Ataxin 3 expression and having any one of the following nucleobase sequences or a nucleobase sequence of 17 contiguous bases contained in the nucleobase sequences: (SEQ?ID?NO:?239) (1) TCGGGTAAGTAGATTTTC, (SEQ?ID?NO:?240) (2) GAAGTATCTGTAGGCCTA, (SEQ?ID?NO:?241) (3) GGACTGTATAGGAGATTA, (SEQ?ID?NO:?242) (4) GGTTATAGGATGCAGGTA, (SEQ?ID?NO:?243) (5) AGGTTATAGGATGCAGGT, (SEQ?ID?NO:?244) (6) GAAGCTAAGTAGGTGACT, (SEQ?ID?NO:?245) (7) TGAAGCTAAGTAGGTGAC, (SEQ?ID?NO:?246) (8) CCTAGTCACTTTGATAGA, (SEQ?ID?NO:?247) (9) GGAACATCTTGAGTAGGT, (SEQ?ID?NO:?248) (10) GGTGTTCAGGGTAGATGT, (SEQ?ID?NO:?249) (11) GGATACTCTGCCCTGTTC, (SEQ?ID?NO:?250) (12) GGTGTCAAACGTGTGGTT, (SEQ?ID?

Abstract: An object of the present invention is to provide a compound, a method and a pharmaceutical composition for normalizing double homeobox 4 (DUX4) of an individual in which the DUX4 gene has abnormally expressed. Provided is a modified oligonucleotide consisting of 12-30 residues. The modified oligonucleotide includes a nucleobase sequence that includes at least 8 contiguous nucleobase sequences and is complementary to an equal length portion at positions 126-147, 232-248, 1306-1325 or 1472-1495 from a 5? end of a nucleobase of a mature mRNA of DUX4 of SEQ ID NO: 1. The nucleobase sequence of the modified oligonucleotide has at least 90% complementarity to the equal length portion in the nucleobase sequence of the mature mRNA of DUX4 of SEQ ID NO: 1.

Abstract: The present invention provides a novel bridged artificial nucleic acid and an oligomer containing the same as a monomer. The present invention provides specifically a compound represented by general formula (I) (wherein each symbol is the same as defined in the specification) or salts thereof; as well as an oligonucleotide compound represented by general formula (I?) (wherein each symbol is the same as defined in the specification) or salts thereof.