Progranulin in frontotemporal lobar degeneration and neuroinflammation

Z Ahmed, IRA Mackenzie, ML Hutton… - Journal of …, 2007 - Springer
Progranulin (PGRN) is a pleiotropic protein that has gained the attention of the neuroscience
community with recent discoveries of mutations in the gene for PGRN that cause …

ALS and FTLD: two faces of TDP‐43 proteinopathy

RM Liscic, LT Grinberg, J Zidar… - European journal of …, 2008 - Wiley Online Library
Major discoveries have been made in the recent past in the genetics, biochemistry and
neuropathology of frontotemporal lobar degeneration (FTLD). TAR DNA‐binding protein 43 …

TDP-43 is a component of ubiquitin-positive tau-negative inclusions in frontotemporal lobar degeneration and amyotrophic lateral sclerosis

T Arai, M Hasegawa, H Akiyama, K Ikeda… - Biochemical and …, 2006 - Elsevier
Ubiquitin-positive tau-negative neuronal cytoplasmic inclusions and dystrophic neurites are
common pathological features in frontotemporal lobar degeneration (FTLD) with or without …

Consensus criteria for the diagnosis of frontotemporal cognitive and behavioural syndromes in amyotrophic lateral sclerosis

MJ Strong, GM Grace, M Freedman… - Amyotrophic Lateral …, 2009 - Taylor & Francis
Amyotrophic lateral sclerosis (ALS) is increasingly recognized to be a multisystem disorder
which includes both clinical and neuropathological features of a frontotemporal lobar …

TDP-43 in familial and sporadic frontotemporal lobar degeneration with ubiquitin inclusions

NJ Cairns, M Neumann, EH Bigio, IE Holm… - The American journal of …, 2007 - Elsevier
TAR DNA-binding protein 43 (TDP-43) is a major pathological protein of sporadic and
familial frontotemporal lobar degeneration with ubiquitin-positive, tau-negative inclusions …

Heterogeneity of ubiquitin pathology in frontotemporal lobar degeneration: classification and relation to clinical phenotype

IRA Mackenzie, A Baborie, S Pickering-Brown… - Acta …, 2006 - Springer
We have investigated the extent and pattern of immunostaining for ubiquitin protein (UBQ) in
60 patients with frontotemporal lobar degeneration (FTLD) with ubiquitin-positive, tau …

The pathological basis of semantic dementia

RR Davies, JR Hodges, JJ Kril, K Patterson… - Brain, 2005 - academic.oup.com
Semantic dementia is a syndrome of progressive deterioration in semantic memory
(knowledge of objects, people, concepts and words). It falls within the clinical spectrum of …

The neuropathology of frontotemporal lobar degeneration caused by mutations in the progranulin gene

IRA Mackenzie, M Baker, S Pickering-Brown… - Brain, 2006 - academic.oup.com
The most common pathology in frontotemporal dementia (FTD) is tau-negative, ubiquitin-
immunoreactive (ub-ir) neuronal inclusions (FTLD-U). Recently, we identified mutations in …

Ubiquitinated pathological lesions in frontotemporal lobar degeneration contain the TAR DNA-binding protein, TDP-43

Y Davidson, T Kelley, IRA Mackenzie… - Acta …, 2007 - Springer
We have investigated the extent and pattern of immunostaining for the TAR DNA-binding
protein, TDP-43, in 37 patients with frontotemporal lobar degeneration with ubiquitin (UBQ) …

TDP-43 is deposited in the Guam parkinsonism–dementia complex brains

M Hasegawa, T Arai, H Akiyama, T Nonaka, H Mori… - Brain, 2007 - academic.oup.com
TDP-43, a nuclear factor that functions in regulating transcription and alternative splicing,
was recently identified as a component of the ubiquitin-positive, tau-negative inclusions …