Selective colocalization of transglutaminase-like activity in ubiquitinated intranuclear inclusions of hereditary dentatorubral-pallidoluysian atrophy

Keiko Sato, Tetsuro Murakami, Yoshiyuki Hamakawa, Hiroshi Kamada, Isao Nagano, Mikio Shoji, Hiroshi Takata, Keigo Nobukuni, Yuetsu Ihara, Reiko Namba, Toshiyuki Hayabara, Shigehisa Hirose, Koji Abe

Research output: Contribution to journalArticlepeer-review

4 Citations (Scopus)

Abstract

To investigate the role of transglutaminase (TG) in the pathophysiology of dentatorubral-pallidoluysian atrophy (DRPLA), the distributions of ubiquitin-positive neuronal intranuclear inclusions (Ub-NII) and TG activity were studied in three patients with DRPLA and four disease controls. In the cerebellar granule cells of DRPLA, 2.5-4.9% of neurons had Ub-NII, and 7.5-9.8% of them were TG positive. In the frontal cortex; however, the ratio of neurons with Ub-NII was relatively low compared with those in the cerebellar cortex, and no Ub-NII was TG positive. There was no distinct difference in the ratio of neurons with Ub-NII and their TG positivity between the cases with homozygous or heterozygous DRPLA patients. The selective and good colocalization of Ub-NII and TG in the cerebellar granule cells may reveal a role of TG in the neurodegenerative process in DRPLA.

Original languageEnglish
Pages (from-to)327-330
Number of pages4
JournalBrain Research
Volume952
Issue number2
DOIs
Publication statusPublished - Oct 18 2002

Keywords

  • Dentatorubral-pallidoluysian atrophy
  • Neuronal intranuclear inclusions
  • Polyglutamine
  • Transglutaminase
  • Ubiquitin

ASJC Scopus subject areas

  • Neuroscience(all)
  • Molecular Biology
  • Clinical Neurology
  • Developmental Biology

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