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 journalArticle

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

Fingerprint

Progressive Myoclonic Epilepsy
Intranuclear Inclusion Bodies
Transglutaminases
Ubiquitin
Neurons
Cerebellar Cortex
Frontal Lobe

Keywords

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

ASJC Scopus subject areas

  • Neuroscience(all)

Cite this

Selective colocalization of transglutaminase-like activity in ubiquitinated intranuclear inclusions of hereditary dentatorubral-pallidoluysian atrophy. / Sato, Keiko; Murakami, Tetsuro; Hamakawa, Yoshiyuki; Kamada, Hiroshi; Nagano, Isao; Shoji, Mikio; Takata, Hiroshi; Nobukuni, Keigo; Ihara, Yuetsu; Namba, Reiko; Hayabara, Toshiyuki; Hirose, Shigehisa; Abe, Koji.

In: Brain Research, Vol. 952, No. 2, 18.10.2002, p. 327-330.

Research output: Contribution to journalArticle

Sato, K, Murakami, T, Hamakawa, Y, Kamada, H, Nagano, I, Shoji, M, Takata, H, Nobukuni, K, Ihara, Y, Namba, R, Hayabara, T, Hirose, S & Abe, K 2002, 'Selective colocalization of transglutaminase-like activity in ubiquitinated intranuclear inclusions of hereditary dentatorubral-pallidoluysian atrophy', Brain Research, vol. 952, no. 2, pp. 327-330. https://doi.org/10.1016/S0006-8993(02)03244-4
Sato, Keiko ; Murakami, Tetsuro ; Hamakawa, Yoshiyuki ; Kamada, Hiroshi ; Nagano, Isao ; Shoji, Mikio ; Takata, Hiroshi ; Nobukuni, Keigo ; Ihara, Yuetsu ; Namba, Reiko ; Hayabara, Toshiyuki ; Hirose, Shigehisa ; Abe, Koji. / Selective colocalization of transglutaminase-like activity in ubiquitinated intranuclear inclusions of hereditary dentatorubral-pallidoluysian atrophy. In: Brain Research. 2002 ; Vol. 952, No. 2. pp. 327-330.
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