Results 31 to 40 of about 201,352 (177)

Frontotemporal Lobar Degeneration and microRNAs

Frontiers in Aging Neuroscience, 2016
Frontotemporal lobar degeneration (FTLD) includes a spectrum of disorders characterized by changes of personality and social behaviour and, often, a gradual and progressive language dysfunction.
Paola ePiscopo, DIEGO eALBANI, Anna Elisa eCastellano, Gianluigi eForloni, Annamaria eConfaloni   +4 more
doaj   +1 more source

Human Ubiquilin 2 and TDP-43 copathology drives neurodegeneration in transgenic Caenorhabditis elegans

G3: Genes, Genomes, Genetics, 2021
Amyotrophic lateral sclerosis (ALS) is a debilitating, fatal neurodegenerative disease that causes rapid muscle wasting. It shares a spectrum of symptoms and pathology with frontotemporal lobar degeneration (FTLD).
Aleen D Saxton, Brian C Kraemer
doaj   +1 more source

Molecular Details of RNA Interactions With FUS Condensates Altering Assembly, Dynamics, and Aggregation

Angewandte Chemie, EarlyView.
Using NMR and molecular simulations, we map how RNA engages FUS LC‐RGG1 within condensates through electrostatic, π‐stacking, and polar contacts. These multivalent RNA contacts help stabilize RNA within FUS condensates while also reshaping condensate composition and dynamics.
Tongyin Zheng, Kandarp A. Sojitra, Samara Cummings, Qizan Chen, Priyesh Mohanty, Jeetain Mittal, Nicolas L. Fawzi   +6 more
wiley   +2 more sources

Clinical, Genetic and Neuropathological Features of Frontotemporal Dementia: An Update and Guide

Acta Médica Portuguesa, 2013
Introduction: Frontotemporal Lobar Degeneration encompasses a group of heterogeneous disorders with shared behavioural and cognitive symptoms, as well as gross pathological features.
Jorge Pelicano Paulos, João Massano
doaj   +1 more source

FOXP2 expression in frontotemporal lobar degeneration-tau [PDF]

, 2020
FOXP2 is altered in a variety of language disorders. We found reduced mRNA and protein expression of FOXP2 in frontal cortex area 8 in Pick's disease, and frontotemporal lobar degeneration-tau linked to P301L mutation presenting with language impairment ...
López González, Irene, Aso Pérez, Ester, Fernandez, Liana, Ferrer, Isidro (Ferrer Abizanda), Carmona Murillo, Margarita, Palmeira, Andre   +5 more
core   +1 more source

Hexanucleotide Repeat Expansion in C9ORF72 Is Not Detected in the Treatment-Resistant Schizophrenia Patients of Chinese Han. [PDF]

PLoS ONE, 2015
Hexanucleotide (GGGGCC) repeat expansion in C9ORF72 (HRE) causes frontotemporal lobar degeneration, frontotemporal dementia-amyotrophic lateral sclerosis, and amyotrophic lateral sclerosis.
Xijia Xu, Shiping Xie, Xiaomeng Shi, Jie Lv, Xiaowei Tang, Xiaolan Wang, Shuiping Lu, Mingzhong Wang, Xiaobing Zhang, Jing Sun, Hui Yao   +10 more
doaj   +1 more source

Mixed neuropathology in frontotemporal lobar degeneration [PDF]

, 2020
Aim: Frontotemporal lobar degeneration (FTLD) is a significant cause of dementia in mid-life and older adults. The extent of interactions between FTLD and other neurodegenerative pathologies is unclear.
Pennington, Catherine; id_orcid, Coulthard, Elizabeth, Love, Seth, Marini, Luca, Pennington, Catherine   +4 more
core   +1 more source

Characterization of Movement Disorder Phenomenology in Genetically Proven, Familial Frontotemporal Lobar Degeneration: A Systematic Review and Meta-Analysis.

PLoS ONE, 2016
BackgroundMutations in granulin (PGRN) and tau (MAPT), and hexanucleotide repeat expansions near the C9orf72 genes are the most prevalent genetic causes of frontotemporal lobar degeneration.
Carmen Gasca-Salas, Mario Masellis, Edwin Khoo, Binit B Shah, David Fisman, Anthony E Lang, Galit Kleiner-Fisman   +6 more
doaj   +1 more source

Response to: Clinical Imaging Features of Sporadic and Genetic Frontotemporal Lobar Degeneration TDP‐43 A and B [PDF]

Annals of Clinical and Translational Neurology
Sergi Borrego‐Écija, Cèlia Cruz‐Escalera, Agnes Perez‐Millán, Jordi Juncà‐Parellà   +3 more
doaj   +2 more sources

Cognitive and Neuroimaging Divergence Between Juvenile and Adult FUS Amyotrophic Lateral Sclerosis

Annals of Clinical and Translational Neurology, EarlyView.
ABSTRACT Objective Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder characterized by progressive motor neuron degeneration. Fused in sarcoma (FUS)‐associated juvenile ALS (jALS) represents a distinct and aggressive subgroup with rapid deterioration and poor prognosis.
Alexandra V. Jürs, Marcel Naumann, Annaliis Lehto, Hanna Schön, Jens Kurth, Johannes Prudlo, Andreas Hermann, Elisabeth Kasper   +7 more
wiley   +1 more source