Advances in understanding the molecular basis of frontotemporal dementia

R Rademakers, M Neumann… - Nature Reviews Neurology, 2012 - nature.com
Frontotemporal dementia (FTD) is a clinical syndrome with a heterogeneous molecular
basis. Until recently, the underlying cause was known in only a minority of cases that were …

Animal models of Alzheimer's disease and frontotemporal dementia

J Götz, LM Ittner - Nature Reviews Neuroscience, 2008 - nature.com
Insoluble protein aggregates have been linked to Alzheimer's disease (AD) and
frontotemporal dementia (FTD). Recent work in transgenic mice has shed light on the role of …

Common variants at 7p21 are associated with frontotemporal lobar degeneration with TDP-43 inclusions

VM Van Deerlin, PMA Sleiman, M Martinez-Lage… - Nature …, 2010 - nature.com
Frontotemporal lobar degeneration (FTLD) is the second most common cause of presenile
dementia. The predominant neuropathology is FTLD with TAR DNA-binding protein (TDP …

The genetics and neuropathology of frontotemporal lobar degeneration

A Sieben, T Van Langenhove, S Engelborghs… - Acta …, 2012 - Springer
Frontotemporal lobar degeneration (FTLD) is a heterogeneous group of disorders
characterized by disturbances of behavior and personality and different types of language …

Alterations of the microRNA network cause neurodegenerative disease

SS Hébert, B De Strooper - Trends in neurosciences, 2009 - cell.com
Brain development crucially depends on the integrity of microRNA (miRNA) pathways, which
function at the post-transcriptional level as a rheostat of the transcriptome and proteome of …

Mutations in α-synuclein, TDP-43 and tau prolong protein half-life through diminished degradation by lysosomal proteases

PJ Sampognaro, S Arya, GM Knudsen… - Molecular …, 2023 - Springer
Background Autosomal dominant mutations in α-synuclein, TDP-43 and tau are thought to
predispose to neurodegeneration by enhancing protein aggregation. While a subset of α …

[PDF][PDF] PGRN is a key adipokine mediating high fat diet-induced insulin resistance and obesity through IL-6 in adipose tissue

T Matsubara, A Mita, K Minami, T Hosooka, S Kitazawa… - Cell metabolism, 2012 - cell.com
Adipose tissue secretes adipokines that mediate insulin resistance, a characteristic feature
of obesity and type 2 diabetes. By differential proteome analysis of cellular models of insulin …

Opposite microglial activation stages upon loss of PGRN or TREM 2 result in reduced cerebral glucose metabolism

JK Götzl, M Brendel, G Werner, S Parhizkar… - EMBO molecular …, 2019 - embopress.org
Microglia adopt numerous fates with homeostatic microglia (HM) and a microglial
neurodegenerative phenotype (MG nD) representing two opposite ends. A number of …

TMEM106B regulates progranulin levels and the penetrance of FTLD in GRN mutation carriers

N Finch, MM Carrasquillo, M Baker, NJ Rutherford… - Neurology, 2011 - AAN Enterprises
Objectives: To determine whether TMEM106B single nucleotide polymorphisms (SNPs) are
associated with frontotemporal lobar degeneration (FTLD) in patients with and without …

FUS pathology defines the majority of tau-and TDP-43-negative frontotemporal lobar degeneration

H Urwin, KA Josephs, JD Rohrer, IR Mackenzie… - Acta …, 2010 - Springer
Through an international consortium, we have collected 37 tau-and TAR DNA-binding
protein 43 (TDP-43)-negative frontotemporal lobar degeneration (FTLD) cases, and present …