Pathogenesis of Frontotemporal Lobar Degeneration: Insights From Loss of Function Theory and Early Involvement of the Caudate Nucleus

Frontotemporal lobar degeneration (FTLD) is a group of clinically, pathologically and genetically heterogeneous neurodegenerative disorders that involve the frontal and temporal lobes. Behavioral variant frontotemporal dementia (bvFTD), semantic dementia (SD), and progressive non-fluent aphasia (PNF...

Full description

Bibliographic Details
Published in:	Frontiers in Neuroscience
Main Authors:	Gen Sobue, Shinsuke Ishigaki, Hirohisa Watanabe
Format:	Article
Language:	English
Published:	Frontiers Media S.A. 2018-07-01
Subjects:	frontotemporal lobar degeneration loss of function tau proteins fused in sarcoma/translated in liposarcoma amyotrophic lateral sclerosis TDP-43 proteinopathies
Online Access:	https://www.frontiersin.org/article/10.3389/fnins.2018.00473/full

Similar Items

Frontotemporal Lobar degeneration with TDP-43 presenting as progressive supranuclear palsy syndrome
by: Aya Murakami, et al.
Published: (2025-07-01)

Increase in wasteosomes (corpora amylacea) in frontotemporal lobar degeneration with specific detection of tau, TDP-43 and FUS pathology
by: Raquel Alsina, et al.
Published: (2024-06-01)

Long noncoding RNAs in TDP-43 and FUS/TLS-related frontotemporal lobar degeneration (FTLD)
by: Guinevere F. Lourenco, et al.
Published: (2015-10-01)

Distinctive autophagy/mitophagy biomarker profiles in frontotemporal lobar degeneration and Alzheimer’s disease
by: Kateřina Veverová, et al.
Published: (2025-02-01)

Simple models to understand complex disease: 10 years of progress from Caenorhabditis elegans models of amyotrophic lateral sclerosis and frontotemporal lobar degeneration
by: Randall J. Eck, et al.
Published: (2024-01-01)

Optogenetic induction of TDP-43 aggregation impairs neuronal integrity and behavior in Caenorhabditis elegans
by: Kyung Hwan Park, et al.
Published: (2025-04-01)

New Perspective on Parkinsonism in Frontotemporal Lobar Degeneration
by: Hee Kyung Park, et al.
Published: (2013-04-01)

Heterogeneity of cortical pTDP-43 inclusion morphologies in amyotrophic lateral sclerosis
by: Rachel H. Tan, et al.
Published: (2023-11-01)

Interrupted CAG expansions in ATXN2 gene expand the genetic spectrum of frontotemporal dementias
by: Clémence Fournier, et al.
Published: (2018-05-01)

Both cytoplasmic and nuclear accumulations of the protein are neurotoxic in Drosophila models of TDP-43 proteinopathies
by: Laetitia Miguel, et al.
Published: (2011-02-01)

Looking into Abnormal Co-Expressions of Tau and TDP-43 in the Realm of Mixed Dementia Types: A Double-Punch Scenario
by: Hossam Youssef, et al.
Published: (2025-07-01)

Pharmacological treatment of frontotemporal lobar degeneration: systematic review Tratamento farmacológico da degeneração lobar frontotemporal: revisão sistemática
by: Maria da Glória Portugal, et al.
Published: (2011-03-01)

Presymptomatic and early pathological features of MAPT-associated frontotemporal lobar degeneration
by: Lucia AA Giannini, et al.
Published: (2023-08-01)

Frontotemporal dementia-like disease progression elicited by seeded aggregation and spread of FUS
by: Sonia Vazquez-Sanchez, et al.
Published: (2024-06-01)

Current understanding of the association between Amyotrophic lateral sclerosis and frontotemporal dementia: Letter to Editor
by: Jie Zhang, et al.
Published: (2023-04-01)

Selective cellular and regional vulnerability in frontotemporal lobar degeneration: a scoping review
by: Kashif Ravasia, et al.
Published: (2025-04-01)

HnRNP Pathologies in Frontotemporal Lobar Degeneration
by: Xinwa Jiang, et al.
Published: (2023-06-01)

Clinical and neuropathological features of ALS/FTD with TIA1 mutations
by: Veronica Hirsch-Reinshagen, et al.
Published: (2017-12-01)

Deletion of the progranulin gene in patients with frontotemporal lobar degeneration or Parkinson disease
by: Anne Rovelet-Lecrux, et al.
Published: (2008-07-01)

Methylome analysis of FTLD patients with TDP-43 pathology identifies epigenetic signatures specific to pathological subtypes
by: Cristina T. Vicente, et al.
Published: (2025-07-01)

The era of cryptic exons: implications for ALS-FTD
by: Puja R. Mehta, et al.
Published: (2023-03-01)

Phosphorylation of Threonine 175 Tau in the Induction of Tau Pathology in Amyotrophic Lateral Sclerosis—Frontotemporal Spectrum Disorder (ALS-FTSD). A Review
by: Alexander J. Moszczynski, et al.
Published: (2018-04-01)

Assessing network degeneration and phenotypic heterogeneity in genetic frontotemporal lobar degeneration by decoding FDG-PET
by: Nick Corriveau-Lecavalier, et al.
Published: (2024-01-01)

Editorial: The role of gene mutations in the neuropathology of amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD)– Progress and challenges
by: Claire Troakes, et al.
Published: (2023-02-01)

Clinical and Imaging Features of Sporadic and Genetic Frontotemporal Lobar Degeneration TDP‐43 A and B
by: Sean Coulborn, et al.
Published: (2025-05-01)

The underappreciated contribution of Oskar Fischer to the knowledge of Frontotemporal Lobar Degeneration
by: Eliasz Engelhardt, et al.
Published: (2025-02-01)

Response to: Clinical Imaging Features of Sporadic and Genetic Frontotemporal Lobar Degeneration TDP‐43 A and B
by: Sergi Borrego‐Écija, et al.
Published: (2025-10-01)

Autoimmunity and Frontotemporal Lobar Degeneration: From Laboratory Study to Clinical Practice
by: Sun Y, et al.
Published: (2023-03-01)

Performance of patients with frontotemporal lobar degeneration on artistic tasks: A pilot study
by: Maria Cristina Anauate, et al.

Parkin Levels Decrease in Fibroblasts With Progranulin (PGRN) Pathogenic Variants and in a Cellular Model of PGRN Deficiency
by: Katarzyna Gaweda-Walerych, et al.
Published: (2021-05-01)

Epigenetic Age Acceleration in Frontotemporal Lobar Degeneration: A Comprehensive Analysis in the Blood and Brain
by: Megha Murthy, et al.
Published: (2023-07-01)

Advances and challenges in understanding the multifaceted pathogenesis of amyotrophic lateral sclerosis
by: Florent Laferriere, et al.
Published: (2015-01-01)

Processing of progranulin into granulins involves multiple lysosomal proteases and is affected in frontotemporal lobar degeneration
by: Swetha Mohan, et al.
Published: (2021-08-01)

CSF neurogranin levels as a biomarker in Alzheimer’s disease and frontotemporal lobar degeneration: a cross-sectional analysis
by: Vanesa Jurasova, et al.
Published: (2024-09-01)

Plasma biomarkers increase diagnostic confidence in patients with Alzheimer’s disease or frontotemporal lobar degeneration
by: Daniele Altomare, et al.
Published: (2024-05-01)

RNA-binding deficient TDP-43 drives cognitive decline in a mouse model of TDP-43 proteinopathy
by: Julie C Necarsulmer, et al.
Published: (2023-10-01)

Diffuse argyrophilic grain disease with TDP-43 proteinopathy and neuronal intermediate filament inclusion disease: FTLD with mixed tau, TDP-43 and FUS pathologies
by: Shunsuke Koga, et al.
Published: (2023-07-01)

DNA methylation as a contributor to dysregulation of STX6 and other frontotemporal Lobar degeneration genetic risk-associated loci
by: Naiomi Rambarack, et al.
Published: (2025-07-01)

Lycorine protects motor neurons against TDP-43 proteinopathy-induced degeneration in cross-species models with amyotrophic lateral sclerosis
by: Jing Wen, et al.
Published: (2024-12-01)

Peripheral proteinopathy in neurodegenerative diseases
by: Bin Xu, et al.
Published: (2025-01-01)