A. Kinoshita et al., UBIQUITIN-RELATED CYTOSKELETAL ABNORMALITY IN FRONTOTEMPORAL DEMENTIA- IMMUNOHISTOCHEMICAL AND IMMUNOELECTRON MICROSCOPE STUDIES, Acta Neuropathologica, 94(1), 1997, pp. 67-72
Although reports of dementia lacking the distinctive non-Alzheimer-typ
e histopathology have been increasing, the concept is still far from c
lear. It has become apparent that this population shows neuropathologi
cal heterogeneity, and some recent reports have proposed a classificat
ion or criteria for these disease conditions. Of the reported cases, f
rontotemporal dementia (FTD) of motor neuron disease is unique in that
the neurons of the hippocampus and entorhinal cortex have ubiquitin-r
elated abnormalities. Recently, a new ubiquitin-related abnormality, c
haracterized by ubiquitinated inclusions in the neurites, has been fou
nd in some FTD cases. Using immunoelectron microscopy with immunogold
particles, we have found that in these two disease conditions ubiquiti
nated inclusions consist of abnormal filaments of 10-15 nm in diameter
. Our results support the speculation that there is a close relationsh
ip between ubiquitin and abnormal filaments in these two types of FTD,
indicating that cytoskeletal-related disorders may underlie certain t
ypes of FTD.