Results 41 to 50 of about 201,352 (177)

Deletion of the progranulin gene in patients with frontotemporal lobar degeneration or Parkinson disease

open access: yesNeurobiology of Disease, 2008
Progranulin gene (PGRN) mutations cause ubiquitin-positive frontotemporal lobar degeneration linked to chromosome 17 (FTLDU-17). The spectrum of known mutations strongly suggests that neurodegeneration results from a partial loss of PGRN function and ...
Anne Rovelet-Lecrux   +10 more
doaj   +1 more source

Prediction and verification of the AD-FTLD common pathomechanism based on dynamic molecular network analysis

open access: yesCommunications Biology, 2021
Jin et al use dynamic molecular network analysis to characterise the phosphoproteome in mouse models of neurodegenerative disease. Analyzing four models of frontotemporal lobar degeneration and four models of Alzheimer’s disease, they observe conserved ...
Meihua Jin   +8 more
doaj   +1 more source

Frontotemporal-TDP and LATE Neurocognitive Disorders: A Pathophysiological and Genetic Approach

open access: yesBrain Sciences, 2023
Frontotemporal lobar degeneration (FTLD) belongs to a heterogeneous group of highly complex neurodegenerative diseases and represents the second cause of presenile dementia in individuals under 65.
Genaro Gabriel Ortiz   +4 more
doaj   +1 more source

Association Between Neurofilament Light Chain and Real‐World Ambulatory Function in Progressive MS

open access: yesAnnals of Clinical and Translational Neurology, EarlyView.
ABSTRACT Objective Neurofilament light chain (NfL) is a biomarker of neuroaxonal injury in multiple sclerosis (MS), yet associations with functional outcomes remain unclear. Longitudinal associations between serum NfL (sNfL) and daily step count (STEPS) from wearable devices were assessed in a large international progressive MS cohort.
Gabby B. Joseph   +5 more
wiley   +1 more source

Neurotransmitter deficits from frontotemporal lobar degeneration. [PDF]

open access: yes, 2018
Frontotemporal lobar degeneration causes a spectrum of complex degenerative disorders including frontotemporal dementia, progressive supranuclear palsy and corticobasal syndrome, each of which is associated with changes in the principal neurotransmitter ...

core   +2 more sources

Progranulin in frontotemporal lobar degeneration and neuroinflammation

open access: yesJournal of Neuroinflammation, 2007
Progranulin (PGRN) is a pleiotropic protein that has gained the attention of the neuroscience community with recent discoveries of mutations in the gene for PGRN that cause frontotemporal lobar degeneration (FTLD).
Hutton Michael L   +3 more
doaj   +1 more source

A Phosphorylation‐Induced Micellization Switch in the Low‐Complexity Domain of TDP‐43

open access: yesAdvanced Science, EarlyView.
Phosphorylation of TAR DNA‐binding protein's 43 kDa (TDP‐43) low‐complexity domain by casein kinase 1 delta (CK1δ) acts as a molecular switch, redirecting its self‐assembly from macroscopic phase separation toward finite‐sized, spherical block‐copolymer micelles of ∼30 nm.
Rodrigo F. Dillenburg   +16 more
wiley   +1 more source

Cerebellar Abnormalities in the Neuroimaging Spectrum of CLTC‐Related Disorder

open access: yesAmerican Journal of Medical Genetics Part A, EarlyView.
ABSTRACT Pathogenic variants in CLTC, which encodes the clathrin heavy chain involved in vesicle‐mediated trafficking in neurons, cause a rare neurodevelopmental disorder associated with variable severity of global developmental delay and intellectual disability and structural brain abnormalities. Although corpus callosum and white matter anomalies are
Daniel Charouf   +7 more
wiley   +1 more source

Apathy and Impulsivity in Frontotemporal Lobar Degeneration Syndromes [PDF]

open access: yes, 2017
There has been considerable progress in the clinical, pathological and genetic fractionation of frontotemporal lobar degeneration syndromes in recent years, driving the development of novel diagnostic criteria.

core   +2 more sources

Cognitive and olfactory function profiles of frontotemporal lobar degeneration

open access: yesChinese Journal of Contemporary Neurology and Neurosurgery, 2021
Objective To describe the cognitive and olfactory function profiles of frontotemporal lobar degeneration (FTLD). Methods A total of 52 FTLD patients admitted to Tianjin Huanhu Hospital from July 2014 to April 2017 were enrolled.
DOU Yu⁃chao   +4 more
doaj   +1 more source

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