Non-Mendelian transmission in dentatorubral-pallidoluysian atrophy and Machado-Joseph disease: the mutant allele is preferentially transmitted in male meiosis.

@article{Ikeuchi1996NonMendelianTI,
  title={Non-Mendelian transmission in dentatorubral-pallidoluysian atrophy and Machado-Joseph disease: the mutant allele is preferentially transmitted in male meiosis.},
  author={Takeshi Ikeuchi and Shuichi Igarashi and Yoshihisa Takiyama and Osamu Onodera and Mutsuo Oyake and Hiroki Takano and Reiji Koide and Hiroyasu Tanaka and Shoji Tsuji},
  journal={American journal of human genetics},
  year={1996},
  volume={58 4},
  pages={
          730-3
        }
}
Autosomal dominant dentatorubral-pallidoluysian atrophy (DRPLA) and Machado-Joseph disease (MJD) are neurodegenerative disorders caused by CAG trinucleotide repeat expansions. An inverse correlation of age at onset with the length of the expanded CAG trinucleotide repeats has been demonstrated, and the intergenerational instability of the length of the CAG trinucleotide repeats, which is more prominent in paternal than in maternal transmissions, has been shown to underlie the basic mechanisms… CONTINUE READING
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