PMID- 24086754
OWN - NLM
STAT- MEDLINE
DCOM- 20140616
LR  - 20211021
IS  - 1932-6203 (Electronic)
IS  - 1932-6203 (Linking)
VI  - 8
IP  - 9
DP  - 2013
TI  - Ubiquilin 2 is not associated with tau pathology.
PG  - e76598
LID - 10.1371/journal.pone.0076598 [doi]
LID - e76598
AB  - Accumulation of aberrant proteins in inclusion bodies is a hallmark of many 
      neurodegenerative diseases. Impairment of proteolytic systems is a common event 
      in these protein misfolding diseases. Recently, mutations in the UBQLN 2 gene 
      encoding ubiquilin 2 have been identified in X-linked amyotrophic lateral 
      sclerosis (ALS). Furthermore, ubiquilin 2 is associated with inclusions in 
      familial and sporadic ALS/dementia, synucleinopathies and polyglutamine diseases. 
      Ubiquilin 2 exerts a regulatory role in proteostasis and thus it has been 
      suggested that ubiquilin 2 pathology may be a common event in neurodegenerative 
      diseases. Tauopathies, a heterogenous group of neurodegenerative diseases 
      accompanied with dementia, are characterized by inclusions of the 
      microtubule-binding protein tau. In the present study, we investigate whether 
      ubiquilin 2 is connected with tau pathology in Alzheimer's disease (AD), 
      supranuclear palsy (PSP) and Pick's disease (PiD) and familial cases with 
      frontotemporal dementia and parkinsonism linked to chromosome 17 (FTDP-17). We 
      show that ubiquilin 2 positive inclusions are absent in these tauopathies. 
      Furthermore, we find decreased ubiquilin 2 protein levels in AD patients, but our 
      results do not indicate a correlation with tau pathology. Our data show no 
      evidence for involvement of ubiquilin 2 and indicate that other mechanisms 
      underly the proteostatic disturbances in tauopathies.
FAU - Nolle, Anna
AU  - Nolle A
AD  - Department of Genome Analysis, Academic Medical Center/University of Amsterdam, 
      Amsterdam, The Netherlands.
FAU - van Haastert, Elise S
AU  - van Haastert ES
FAU - Zwart, Rob
AU  - Zwart R
FAU - Hoozemans, Jeroen J M
AU  - Hoozemans JJ
FAU - Scheper, Wiep
AU  - Scheper W
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
DEP - 20130926
PL  - United States
TA  - PLoS One
JT  - PloS one
JID - 101285081
RN  - 0 (Adaptor Proteins, Signal Transducing)
RN  - 0 (Autophagy-Related Proteins)
RN  - 0 (Cell Cycle Proteins)
RN  - 0 (UBQLN2 protein, human)
RN  - 0 (Ubiquitins)
RN  - 0 (tau Proteins)
SB  - IM
MH  - Adaptor Proteins, Signal Transducing
MH  - Aged
MH  - Aged, 80 and over
MH  - Autophagy-Related Proteins
MH  - Cell Cycle Proteins/*metabolism
MH  - Cell Line, Tumor
MH  - Humans
MH  - Middle Aged
MH  - Neurodegenerative Diseases/*metabolism/*pathology
MH  - Ubiquitins/*metabolism
MH  - tau Proteins/*metabolism
PMC - PMC3784422
COIS- Competing Interests: The authors have declared that no competing interests exist.
EDAT- 2013/10/03 06:00
MHDA- 2014/06/17 06:00
PMCR- 2013/09/26
CRDT- 2013/10/03 06:00
PHST- 2013/06/07 00:00 [received]
PHST- 2013/09/03 00:00 [accepted]
PHST- 2013/10/03 06:00 [entrez]
PHST- 2013/10/03 06:00 [pubmed]
PHST- 2014/06/17 06:00 [medline]
PHST- 2013/09/26 00:00 [pmc-release]
AID - PONE-D-13-23099 [pii]
AID - 10.1371/journal.pone.0076598 [doi]
PST - epublish
SO  - PLoS One. 2013 Sep 26;8(9):e76598. doi: 10.1371/journal.pone.0076598. eCollection 
      2013.