Failure in heat-shock protein expression in response to UBB+1 protein in progressive supranuclear palsy in humans.
UBB+1 protein is an aberrant ubiquitin associated with progressive supranuclear palsy (PSP). It leads to proteasome inhibition, heat-shock protein (HSP) expression and apoptosis in cell cultures. Despite UBB+1 polyubiquitination (an indication of proteasome inhibition), we demonstrate that UBB+1 and HSP40/HSP70 immunoreactivity do not co-localize in the pons of patients with PSP. As HSPs are involved in both normal tau and proteasome function, these findings may be relevant to the aetiology of PSP and other tauopathies.
Hope
Andrew D
AD
Reta Lila Weston Institute of Neurological Studies, University College London, 46 Cleveland Street, London, W1T 4JF, UK. hope.andrew@mayo.edu
Lashley
Tammaryn
T
Lees
Andrew J
AJ
de Silva
Rohan
R
D000368:Aged; D000369:Aged, 80 and over; D005260:Female; D005786:Gene Expression Regulation / Q000502:physiology*; D006360:Heat-Shock Proteins / Q000096:biosynthesis* / Q000235:genetics; D006801:Humans; D008297:Male; D008875:Middle Aged; D011149:Pons / Q000378:metabolism* / Q000473:pathology; D013494:Supranuclear Palsy, Progressive / Q000378:metabolism* / Q000473:pathology; D025801:Ubiquitin / Q000096:biosynthesis* / Q000235:genetics
D006360:Heat-Shock Proteins; C479419:UBB protein, human; D025801:Ubiquitin