Frontotemporal lobar degeneration (FTLD) is a group of complex neurodegenerative disease characterized by progressive deterioration of the frontal and anterior temporal lobes of the brain resulting in different heterogeneous conditions, mainly characterized by personality changes, behavioral disturbances, such as binge eating, and deficits in language and executive functions. Null mutations in progranulin gene (GRN) are one of the most frequent genetic determinants in familial frontotemporal dementia. Recently, progranulin was recognized as an adipokine involved in diet-induced obesity and insulin resistance revealing its metabolic function. Increasing evidence suggests that neurodegenerative dementias are associated with a higher prevalence of metabolic changes than in the general population. According to these findings, the aim of this study is to investigate putative alterations in markers linked to metabolic functions (i.e., C-peptide, ghrelin, glucose-dependent insulinotropic polypeptide, glucagon-like peptide-1, glucagon, insulin, resistin, and three adipokines as visfatin, leptin, and plasminogen activator inhibitor-1 total) in sporadic and GRN-related FTLD. We found that 1) C-peptide is increased in sporadic and GRN-mutated FTLD patients; in addition, we demonstrated an anticipation of the disease in patients with the highest C-peptide concentrations; 2) visfatin is slightly reduced in the whole FTLD group; 3) resistin, an adipokine involved in inflammatory-related diseases, is specifically increased in FTLD due to GRN null mutations; 4) ghrelin concentration is specifically increased in pre-symptomatic subjects and FTLD patients with GRN mutations. These findings support the hypothesis that alterations in metabolic pattern are involved in FTLD progression highlighting novel putative targets for the development of preventive and personalized therapies.

Download full-text PDF

Source
http://dx.doi.org/10.3233/JAD-170747DOI Listing

Publication Analysis

Top Keywords

frontotemporal lobar
8
lobar degeneration
8
ftld group
8
null mutations
8
adipokine involved
8
ftld patients
8
ftld
7
serum c-peptide
4
c-peptide visfatin
4
visfatin resistin
4

Similar Publications

Background: TAR-DNA-binding protein 43 (TDP43), is a pathologic marker in neurodegenerative diseases including frontotemporal lobar degeneration and amyotrophic lateral sclerosis. The aggregation of TDP-43, a crucial RNA-binding protein, is a consequence of post-translational modifications (PTMs) that disrupt its normal function. PTMs such as phosphorylation and ubiquitination contribute to the aberrant accumulation of TDP-43 aggregates, leading to neurodegenerative disorders like amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD).

View Article and Find Full Text PDF

Dementia Care Research and Psychosocial Factors.

Alzheimers Dement

December 2024

Memory and Aging Center, UCSF Weill Institute for Neurosciences, University of California, San Francisco, San Francisco, CA, USA.

Background: Many treatments targeting frontotemporal lobar degeneration (FTLD) are in the developmental pipeline, but the rarity of the disease, coupled with the behavioral and motor features of FTLD, make it challenging to identify sufficient trial participants who can attend frequent in-person visits. Decentralized clinical trial designs with remote evaluations are attractive alternatives but require validated tools for symptom tracking. Our previous cross-sectional analyses showed that cognitive tasks deployed via the ALLFTD Mobile App are reliable and sensitive to early stages of disease.

View Article and Find Full Text PDF

Technology and Dementia Preconference.

Alzheimers Dement

December 2024

UT Health San Antonio, San Antonio, TX, USA.

Background: Primary progressive aphasia (PPA) is a language-led dementia associated with underlying Alzheimer's disease (AD) or frontotemporal lobar degeneration pathology. As part of the Alzheimer's spectrum, logopenic (lv) PPA may be particularly difficult to distinguish from amnestic AD, due to overlapping clinical features. Analysis of linguistic and acoustic variables derived from connected speech has shown promise as a diagnostic tool for differentiating dementia subtypes.

View Article and Find Full Text PDF

Introduction: Plasma-based biomarkers have shown promise for clinical implementation, but their accuracy in differentiating Alzheimer's disease (AD) from syndromes associated with frontotemporal lobar degeneration (FTLD) has yet to be fully investigated. This study assessed the potential of plasma biomarkers for differential diagnosis.

Methods: This cohort study included 374 participants (96 AD, 278 FTLD).

View Article and Find Full Text PDF

Screening properties of the updated normative framework for the Italian MMSE in MCI and dementia.

Neurol Sci

January 2025

Department of Neurology and Laboratory of Neuroscience, IRCCS Istituto Auxologico Italiano, Milano, Italy.

Background: This study aimed to assess the screening properties of Foderaro et al.s' updated normative framework for the Italian MMSE in detecting mild cognitive impairment (MCI) and dementia due to neurodegenerative, chronic cerebrovascular, and mixed etiologies, as well as in differentiating between these two syndromes.

Methods: Data on 234 patients with either MCI (N = 83) or dementia (N = 151) due to Alzheimer's disease (N = 112), Lewy body disease (N = 11), frontotemporal lobar degeneration (N = 20), chronic cerebrovascular disease (N = 39), or mixed (N = 47) etiologies having been administered Foderaro et al.

View Article and Find Full Text PDF

Want AI Summaries of new PubMed Abstracts delivered to your In-box?

Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!