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Human Genetics

, Volume 107, Issue 5, pp 452–457 | Cite as

Differential somatic CAG repeat instability in variable brain cell lineage in dentatorubral pallidoluysian atrophy (DRPLA): a laser-captured microdissection (LCM)-based analysis

  • Hidetaka Watanabe
  • Fumiaki Tanaka
  • Manabu Doyu
  • Shigeo Riku
  • Mari Yoshida
  • Yoshio Hashizume
  • Gen Sobue
Original Investigation

Abstract.

Employing a laser-captured microdissection (LCM), we have investigated the somatic instability of CAG repeats in the variable brain cell lineage in three patients with dentatorubral pallidoluysian atrophy (DRPLA). LCM enables the isolation of single lineage brain cells for subsequent molecular analysis. We have found that CAG repeat size and the range of CAG repeats in the cerebellar granular cells is smaller than those in cerebellar glial cells. Similarly, those in the cerebral neuronal cells are significantly shorter than those in cerebral glial cells. These data directly indicate that the CAG repeat is relatively more stable in neuronal cells than in glial cells. Furthermore, cerebellar granular cells show significantly smaller main CAG repeat size and CAG repeat range than either Purkinje cells or cerebral neuronal cells, suggesting that somatic instability in the CAG repeat is markedly variable even among the different types of neuronal populations. The cell-specific CAG repeat instability may thus be more complex than has previously been considered. LCM is a powerful tool for elucidating the mechanism of the triplet repeat instability of each cell type.

Keywords

Glial Cell Purkinje Cell Brain Cell Neuronal Population Triplet Repeat 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

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Copyright information

© Springer-Verlag 2000

Authors and Affiliations

  • Hidetaka Watanabe
    • 1
  • Fumiaki Tanaka
    • 1
  • Manabu Doyu
    • 1
  • Shigeo Riku
    • 2
  • Mari Yoshida
    • 3
  • Yoshio Hashizume
    • 3
  • Gen Sobue
    • 1
  1. 1.Department of Neurology, Nagoya University School of Medicine, 65 Tsurumai-cho, Showa-ku, Nagoya 466–8550, Japan
  2. 2.Department of Neurology, Chukyo Hospital, Nagoya, Japan
  3. 3.Institute for Medical Science of Aging, Aichi Medical University, Aichi, Japan

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