title: Aberrantly regulated proteins in frontotemporal dementia.
abstract: Non-Alzheimer's disease of the frontal type, or frontotemporal dementia (FTD), is the second most common form of dementia. Yet, a detailed characterization of the disease has been especially limiting. To identify mechanisms possibly involved in disease pathology or progression, a proteomic analysis of proteins isolated from human frontal cortex with frontotemporal dementia and parkinsonism linked to chromosome 17 (FTDP-17) was performed. We used 2D gel electrophoresis and MALDI-TOF to identify a total of 24 proteins differentially expressed in FTDP-17. We identified a ubiquitin C-terminal hydrolase, UCHL1, as well as several proteins involved in oxidative stress to be differentially expressed. Data presented implicate UCHL1 and ubiquitin-mediated degradation as well as oxidative stress response in disease pathology or progression.
authors.affiliation: Department of Pathology and Laboratory Medicine, Indiana University School of Medicine, Indianapolis, IN 46202, USA. kschwei2@jhmi.edu
authors.forename: Kelly
authors.initials: K
authors.lastname: Schweitzer
authors.forename: Emily
authors.initials: E
authors.lastname: Decker
authors.forename: Liping
authors.initials: L
authors.lastname: Zhu
authors.forename: Richard E
authors.initials: RE
authors.lastname: Miller
authors.forename: Suzanne S
authors.initials: SS
authors.lastname: Mirra
authors.forename: Salvatore
authors.initials: S
authors.lastname: Spina
authors.forename: Bernardino
authors.initials: B
authors.lastname: Ghetti
authors.forename: Mu
authors.initials: M
authors.lastname: Wang
authors.forename: Jill
authors.initials: J
authors.lastname: Murrell
mesh_terms: D002886:Chromosomes, Human, Pair 17 / Q000235:genetics; D003704:Dementia / Q000503:physiopathology*; D015180:Electrophoresis, Gel, Two-Dimensional; D005260:Female; D005293:Ferritins / Q000378:metabolism; D005625:Frontal Lobe / Q000737:chemistry / Q000378:metabolism; D006801:Humans; D008297:Male; D008875:Middle Aged; D009419:Nerve Tissue Proteins / Q000378:metabolism*; D018384:Oxidative Stress / Q000235:genetics; D040901:Proteomics; D019032:Spectrometry, Mass, Matrix-Assisted Laser Desorption-Ionization; D025801:Ubiquitin / Q000378:metabolism; D043222:Ubiquitin Thiolesterase / Q000235:genetics
chemical_list: D009419:Nerve Tissue Proteins; C482860:UCHL1 protein, human; D025801:Ubiquitin; D005293:Ferritins; D043222:Ubiquitin Thiolesterase