Aggregate distribution in frontal and motor cortex in Huntington's disease brain.
Insoluble protein aggregates have been considered a pathological hallmark of Huntington's disease and other polyglutamine disorders. In this study the number of aggregates was assessed in the superior frontal gyrus and motor cortex of seven Huntington's disease patients and was compared with the symptoms (motor/mood) these patients displayed during the course of the disease. Regardless of the pattern of symptoms present in the patients, there was a consistently higher number of nuclear and non-nuclear aggregates in the superior frontal gyrus than in the motor cortex. This suggests that there is a consistent regional difference in the density of aggregates and that this consistency is not reflected in the variable symptomatology between cases.
van Roon-Mom
Willeke M C
WM
Department of Anatomy with Radiology, University of Auckland, Auckland, New Zealand. w.van_roon@lumc.nl
Hogg
Virginia M
VM
Tippett
Lynette J
LJ
Faull
Richard L M
RL
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D002467:Cell Nucleus / Q000378:metabolism / Q000473:pathology; D005260:Female; D005625:Frontal Lobe / Q000378:metabolism*; D006801:Humans; D000071058:Huntingtin Protein; D006816:Huntington Disease / Q000378:metabolism / Q000473:pathology* / Q000503:physiopathology; D007150:Immunohistochemistry / Q000379:methods; D008297:Male; D008875:Middle Aged; D009044:Motor Cortex / Q000378:metabolism*; D009419:Nerve Tissue Proteins / Q000378:metabolism*; D009687:Nuclear Proteins / Q000378:metabolism*
C086055:HTT protein, human; D000071058:Huntingtin Protein; D009419:Nerve Tissue Proteins; D009687:Nuclear Proteins