TY - JOUR
T1 - Selective colocalization of transglutaminase-like activity in ubiquitinated intranuclear inclusions of hereditary dentatorubral-pallidoluysian atrophy
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.
KW - Dentatorubral-pallidoluysian atrophy
KW - Neuronal intranuclear inclusions
KW - Polyglutamine
KW - Transglutaminase
KW - Ubiquitin
UR - http://www.scopus.com/inward/record.url?scp=0037131081&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=0037131081&partnerID=8YFLogxK
U2 - 10.1016/S0006-8993(02)03244-4
DO - 10.1016/S0006-8993(02)03244-4
M3 - Article
C2 - 12376195
AN - SCOPUS:0037131081
VL - 952
SP - 327
EP - 330
JO - Molecular Brain Research
JF - Molecular Brain Research
SN - 0006-8993
IS - 2
ER -