Abstract:
:Incorporation of ubiquitin and ubiquitin-related proteins including p62 into neuronal intranuclear inclusions (NIIs) has been reported in a variety of neurodegenerative diseases. However, involvement of autophagy-specific proteins (NBR1 and LC3) in NIIs has not been mentioned. We immunohistochemically examined the brain of patients with Machado-Joseph disease (MJD; n=5), dentatorubral-pallidoluysian atrophy (DRPLA; n=5) and intranuclear inclusion body disease (INIBD; n=5), using antibodies against ubiquitin, p62, NBR1 and LC3. The proportion of p62-, NBR1- and LC3-positive inclusions relative to the number of ubiquitin-positive inclusions was calculated in each case. NIIs were positive for p62 in MJD (19.3%), DRPLA (49.7%) and INIBD (99.8%). As for autophagy-specific proteins, NIIs were positive for NBR1 in MJD (4.2%), DRPLA (5.5%) and INIBD (13.2%) and negative for LC3 in MJD, DRPLA and INIBD, except for one case of INIBD. These findings suggest that autophagy-lysosome pathway is not involved in the formation/degradation of NIIs.
journal_name
Neurosci Lettjournal_title
Neuroscience lettersauthors
Mori F,Tanji K,Odagiri S,Toyoshima Y,Yoshida M,Kakita A,Takahashi H,Wakabayashi Kdoi
10.1016/j.neulet.2012.06.026subject
Has Abstractpub_date
2012-08-01 00:00:00pages
134-8issue
2eissn
0304-3940issn
1872-7972pii
S0304-3940(12)00824-5journal_volume
522pub_type
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