Results 101 to 110 of about 9,658 (171)
Abstract INTRODUCTION The impact of different neuropathologies on deep brain structures remains to be understood. We examine subcortical and limbic volumetry in neurodegenerative diseases involving phosphorylated tau (p‐tau), α‐synuclein, and transactive response DNA binding protein 43 (TDP‐43).
Pulkit Khandelwal +30 more
wiley +1 more source
Nuclear dysfunction in aging and neurodegeneration
Abstract Neurodegenerative diseases are characterized by a loss of neuronal function and structure, often in a region‐specific manner. Multiple factors contribute to neuronal dysfunction and death, including pathogenic protein buildup, protein mislocalization, and inflammation. Despite extensive research, the common mechanisms driving neurodegeneration
Abbigael Aday +7 more
wiley +1 more source
Objective Immunohistochemically (IHC) measured transactive response DNA‐binding protein 43 (TDP‐43) inclusions are observed in Alzheimer's disease (AD) and are associated with medial temporal lobe atrophy. Accumulation of cryptic exons occurs in AD in response to TDP‐43 pathology.
Hossam Youssef +18 more
wiley +1 more source
ABSTRACT Although much is known about the encoding of experience, how the brain organizes neural circuits capable of learning and memory formation is largely unstudied. Canonical critical periods emerge from a convergence of maturation‐ and experience‐dependent processes.
Grant W. Kunzelman +2 more
wiley +1 more source
C9orf72 plays a central role in Rab GTPase-dependent regulation of autophagy [PDF]
A GGGGCC hexanucleotide repeat expansion in the first intron of the C9orf72 gene is the most common genetic defect associated with amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) (C9ALS/FTD).
De Vos, K. +3 more
core +1 more source
Plasma Proteomic Changes in GRN and C9orf72 Frontotemporal Dementia
Multiplex plasma profiling of 124 CNS‐related proteins in genetic frontotemporal dementia (FTD) identified NfL and NfH as the most consistently altered biomarkers in both GRN‐ and C9orf72‐associated disease. Distinct protein changes emerged across genotypes, including changes in GFAP and VCAM1 in GRN‐FTD, and reduced NPTXR as well as nominally altered ...
Joel Simrén +11 more
wiley +1 more source
Amyotrophic lateral sclerosis (ALS) involves widespread cortical pathology beyond the motor cortex. Human‐induced pluripotent stem cell‐derived neural organoids model cortical tissue in vitro and provide a physiologically relevant platform to study disease mechanisms in ALS.
Kristel N. Eigenhuis +2 more
wiley +1 more source
Background: TDP-43 is the main protein component of ubiquitinated inclusions in a subgroup of frontotemporal lobar degeneration (FTLD) and amyotrophic lateral sclerosis (ALS) patients.
Anna Junttila +10 more
doaj +1 more source
Background Amyotrophic Lateral Sclerosis (ALS), a neurodegenerative disease involving loss of motor neurons, typically results in death within 3–5 years of disease onset. Although roughly 10% of cases can be linked to a specific inherited mutation (e.g.,
Adam N. Trautwig +24 more
doaj +1 more source
Cytoplasmic aggregates of the predominantly nuclear TAR DNA-binding protein 43 (TDP-43) are a pathological hallmark of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) cases caused by G4C2 hexanucleotide repeat expansions in C9orf72 (
Lilian Tsai-Wei Lin +4 more
doaj +1 more source

