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

doi:10.1016/S0006-8993(02)03244-4

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 journal › Article › peer-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 language	English
Pages (from-to)	327-330
Number of pages	4
Journal	Brain Research
Volume	952
Issue number	2
DOIs	https://doi.org/10.1016/S0006-8993(02)03244-4
Publication status	Published - 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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10.1016/S0006-8993(02)03244-4

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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, 952(2), 327-330. https://doi.org/10.1016/S0006-8993(02)03244-4

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 journal › Article › peer-review

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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title = "Selective colocalization of transglutaminase-like activity in ubiquitinated intranuclear inclusions of hereditary dentatorubral-pallidoluysian atrophy",

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.",

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note = "Funding Information: We thank Dr. Y. Furutani for the technical advice for the present study. This work was partly supported by Grant-in-Aid for Scientific Research (B) 12470141 from the Ministry of Education, Science, Culture and Sports of Japan, and by grants (Tashiro K, Itoyama Y and Tsuji S) and Comprehensive Research on Aging and Health (H11-Choju-010, No.207, Koizumi A) from the Ministry of Health and Welfare of Japan.",

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AU - Sato, Keiko

AU - Murakami, Tetsuro

AU - Hamakawa, Yoshiyuki

AU - Kamada, Hiroshi

AU - Nagano, Isao

AU - Shoji, Mikio

AU - Takata, Hiroshi

AU - Nobukuni, Keigo

AU - Ihara, Yuetsu

AU - Namba, Reiko

AU - Hayabara, Toshiyuki

AU - Hirose, Shigehisa

AU - Abe, Koji

N1 - Funding Information: We thank Dr. Y. Furutani for the technical advice for the present study. This work was partly supported by Grant-in-Aid for Scientific Research (B) 12470141 from the Ministry of Education, Science, Culture and Sports of Japan, and by grants (Tashiro K, Itoyama Y and Tsuji S) and Comprehensive Research on Aging and Health (H11-Choju-010, No.207, Koizumi A) from the Ministry of Health and Welfare of Japan.

PY - 2002/10/18

Y1 - 2002/10/18

N2 - 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.

AB - 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.

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

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