Frontotemporal dementia

J Bang, S Spina, BL Miller - The Lancet, 2015 - thelancet.com
Frontotemporal dementia is an umbrella clinical term that encompasses a group of
neurodegenerative diseases characterised by progressive deficits in behaviour, executive …

Progranulin, lysosomal regulation and neurodegenerative disease

AW Kao, A McKay, PP Singh, A Brunet… - Nature Reviews …, 2017 - nature.com
The discovery that heterozygous and homozygous mutations in the gene encoding
progranulin are causally linked to frontotemporal dementia and lysosomal storage disease …

An autophagic flux probe that releases an internal control

T Kaizuka, H Morishita, Y Hama, S Tsukamoto… - Molecular cell, 2016 - cell.com
Macroautophagy is an intracellular degradation system that utilizes the autophagosome to
deliver cytoplasmic components to the lysosome. Measuring autophagic activity is critically …

TREM2 mutations implicated in neurodegeneration impair cell surface transport and phagocytosis

G Kleinberger, Y Yamanishi, M Suárez-Calvet… - Science translational …, 2014 - science.org
Genetic variants in the triggering receptor expressed on myeloid cells 2 (TREM2) have been
linked to Nasu-Hakola disease, Alzheimer's disease (AD), Parkinson's disease, amyotrophic …

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 …

[HTML][HTML] Progranulin deficiency promotes neuroinflammation and neuron loss following toxin-induced injury

LH Martens, J Zhang, SJ Barmada… - The Journal of …, 2012 - Am Soc Clin Investig
Progranulin (PGRN) is a widely expressed secreted protein that is linked to inflammation. In
humans, PGRN haploinsufficiency is a major inherited cause of frontotemporal dementia …

Development of disease-modifying drugs for frontotemporal dementia spectrum disorders

F Panza, M Lozupone, D Seripa, A Daniele… - Nature Reviews …, 2020 - nature.com
Frontotemporal dementia (FTD) encompasses a spectrum of clinical syndromes
characterized by progressive executive, behavioural and language dysfunction. The various …

Microglia and astrocyte involvement in neurodegeneration and brain cancer

AA Vandenbark, H Offner, S Matejuk… - Journal of …, 2021 - Springer
The brain is unique and the most complex organ of the body, containing neurons and
several types of glial cells of different origins and properties that protect and ensure normal …

TMEM106B, the risk gene for frontotemporal dementia, is regulated by the microRNA-132/212 cluster and affects progranulin pathways

AS Chen-Plotkin, TL Unger, MD Gallagher… - Journal of …, 2012 - Soc Neuroscience
Frontotemporal lobar degeneration with TDP-43 inclusions (FTLD-TDP) is a fatal
neurodegenerative disease with no available treatments. Mutations in the progranulin gene …

Progranulin gene therapy improves lysosomal dysfunction and microglial pathology associated with frontotemporal dementia and neuronal ceroid lipofuscinosis

AE Arrant, VC Onyilo, DE Unger… - Journal of …, 2018 - Soc Neuroscience
Loss-of-function mutations in progranulin, a lysosomal glycoprotein, cause
neurodegenerative disease. Progranulin haploinsufficiency causes frontotemporal dementia …