[HTML][HTML] Lysosome dysfunction as a cause of neurodegenerative diseases: Lessons from frontotemporal dementia and amyotrophic lateral sclerosis

J Root, P Merino, A Nuckols, M Johnson… - Neurobiology of disease, 2021 - Elsevier
Frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS) are fatal
neurodegenerative disorders that are thought to exist on a clinical and pathological …

Repositioning of anti-diabetic drugs against dementia: insight from molecular perspectives to clinical trials

KEK Mantik, S Kim, B Gu, S Moon, HB Kwak… - International journal of …, 2023 - mdpi.com
Insulin resistance as a hallmark of type 2 DM (T2DM) plays a role in dementia by promoting
pathological lesions or enhancing the vulnerability of the brain. Numerous studies related to …

[HTML][HTML] Rescue of a lysosomal storage disorder caused by Grn loss of function with a brain penetrant progranulin biologic

T Logan, MJ Simon, A Rana, GM Cherf, A Srivastava… - Cell, 2021 - cell.com
GRN mutations cause frontotemporal dementia (GRN-FTD) due to deficiency in progranulin
(PGRN), a lysosomal and secreted protein with unclear function. Here, we found that Grn …

Network analysis of the progranulin-deficient mouse brain proteome reveals pathogenic mechanisms shared in human frontotemporal dementia caused by GRN …

M Huang, E Modeste, E Dammer, P Merino… - Acta neuropathologica …, 2020 - Springer
Heterozygous, loss-of-function mutations in the granulin gene (GRN) encoding progranulin
(PGRN) are a common cause of frontotemporal dementia (FTD). Homozygous GRN …

[HTML][HTML] Neuroimaging in frontotemporal dementia: heterogeneity and relationships with underlying neuropathology

BT Peet, S Spina, N Mundada, R La Joie - Neurotherapeutics, 2021 - Elsevier
Frontotemporal dementia encompasses a group of clinical syndromes defined
pathologically by degeneration of the frontal and temporal lobes. Historically, these …

Advances in treatment of frontotemporal dementia

N Magrath Guimet… - The Journal of …, 2022 - Am Neuropsych Assoc
In this review, the authors explored the clinical features of frontotemporal dementia (FTD),
focusing on treatment. The clinical features of FTD are unique, with disinhibition, apathy …

Primary progressive aphasia: a model for neurodegenerative disease

BL Tee, ML Gorno-Tempini - Current opinion in neurology, 2019 - journals.lww.com
Primary progressive aphasia: a model for neurodegenerative d... : Current Opinion in
Neurology Primary progressive aphasia: a model for neurodegenerative disease : Current …

Genetics and molecular mechanisms of frontotemporal lobar degeneration: an update and future avenues

R Ferrari, C Manzoni, J Hardy - Neurobiology of aging, 2019 - Elsevier
Frontotemporal lobar degeneration (FTLD) is the second most common form of dementia
after Alzheimer's disease. The study and the dissection of FTLD is complex due to its clinical …

Karyopherin abnormalities in neurodegenerative proteinopathies

T Pasha, A Zatorska, D Sharipov, B Rogelj… - Brain, 2021 - academic.oup.com
Neurodegenerative proteinopathies are characterized by progressive cell loss that is
preceded by the mislocalization and aberrant accumulation of proteins prone to …

Recent advances in the genetics of frontotemporal dementia

DW Sirkis, EG Geier, LW Bonham, CM Karch… - … genetic medicine reports, 2019 - Springer
Abstract Purpose of Review In this review, we highlight recent advances in the human
genetics of frontotemporal dementia (FTD). In addition to providing a broad survey of genes …