Selective colocalization of transglutaminase-like activity in ubiquitinated intranuclear inclusions of hereditary dentatorubral-pallidoluysian atrophy.
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.
Sato
Keiko
K
Department of Neurology, Graduate School of Medicine and Dentistry, Okayama University, 2-5-1 Shikata-cho, Okayama 700-8558, Japan. satokei@cc.okayama-u.ac.jp
Murakami
Tetsuro
T
Hamakawa
Yoshiyuki
Y
Kamada
Hiroshi
H
Nagano
Isao
I
Shoji
Mikio
M
Takata
Hiroshi
H
Nobukuni
Keigo
K
Ihara
Yuetsu
Y
Namba
Reiko
R
Hayabara
Toshiyuki
T
Hirose
Shigehisa
S
Abe
Koji
K
D000328:Adult; D000368:Aged; D002525:Cerebellar Cortex / Q000737:chemistry / Q000201:enzymology / Q000473:pathology; D002540:Cerebral Cortex / Q000737:chemistry / Q000201:enzymology / Q000473:pathology; D005260:Female; D006801:Humans; D002479:Inclusion Bodies / Q000737:chemistry / Q000378:metabolism / Q000473:pathology; D008297:Male; D008875:Middle Aged; D020191:Myoclonic Epilepsies, Progressive / Q000201:enzymology* / Q000473:pathology; D009474:Neurons / Q000737:chemistry / Q000201:enzymology / Q000473:pathology; D011503:Transglutaminases / Q000032:analysis* / Q000378:metabolism; D025801:Ubiquitin / Q000032:analysis* / Q000378:metabolism
D025801:Ubiquitin; D011503:Transglutaminases