PMID- 16890190 OWN - NLM STAT- MEDLINE DCOM- 20061010 LR - 20190402 IS - 0006-291X (Print) IS - 0006-291X (Linking) VI - 348 IP - 2 DP - 2006 Sep 22 TI - Aberrantly regulated proteins in frontotemporal dementia. PG - 465-72 AB - 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. FAU - Schweitzer, Kelly AU - Schweitzer K AD - Department of Pathology and Laboratory Medicine, Indiana University School of Medicine, Indianapolis, IN 46202, USA. kschwei2@jhmi.edu FAU - Decker, Emily AU - Decker E FAU - Zhu, Liping AU - Zhu L FAU - Miller, Richard E AU - Miller RE FAU - Mirra, Suzanne S AU - Mirra SS FAU - Spina, Salvatore AU - Spina S FAU - Ghetti, Bernardino AU - Ghetti B FAU - Wang, Mu AU - Wang M FAU - Murrell, Jill AU - Murrell J LA - eng GR - P30 AG10133/AG/NIA NIH HHS/United States GR - R01 NS37431/NS/NINDS NIH HHS/United States PT - Journal Article PT - Research Support, N.I.H., Extramural DEP - 20060728 PL - United States TA - Biochem Biophys Res Commun JT - Biochemical and biophysical research communications JID - 0372516 RN - 0 (Nerve Tissue Proteins) RN - 0 (UCHL1 protein, human) RN - 0 (Ubiquitin) RN - 9007-73-2 (Ferritins) RN - EC 3.4.19.12 (Ubiquitin Thiolesterase) SB - IM MH - Chromosomes, Human, Pair 17/genetics MH - Dementia/*physiopathology MH - Electrophoresis, Gel, Two-Dimensional MH - Female MH - Ferritins/metabolism MH - Frontal Lobe/chemistry/metabolism MH - Humans MH - Male MH - Middle Aged MH - Nerve Tissue Proteins/*metabolism MH - Oxidative Stress/genetics MH - Proteomics MH - Spectrometry, Mass, Matrix-Assisted Laser Desorption-Ionization MH - Ubiquitin/metabolism MH - Ubiquitin Thiolesterase/genetics EDAT- 2006/08/08 09:00 MHDA- 2006/10/13 09:00 CRDT- 2006/08/08 09:00 PHST- 2006/06/29 00:00 [received] PHST- 2006/07/17 00:00 [accepted] PHST- 2006/08/08 09:00 [pubmed] PHST- 2006/10/13 09:00 [medline] PHST- 2006/08/08 09:00 [entrez] AID - S0006-291X(06)01623-8 [pii] AID - 10.1016/j.bbrc.2006.07.113 [doi] PST - ppublish SO - Biochem Biophys Res Commun. 2006 Sep 22;348(2):465-72. doi: 10.1016/j.bbrc.2006.07.113. Epub 2006 Jul 28.