TDP-43 Nuclear Loss in FTD/ALS Causes Widespread Alternative Polyadenylation Changes

Medicine, Basic Medical Sciences, Neurology, Life Sciences, Cell Biology, Biochemistry, Genetics,
TDP-43   frontotemporal dementia   amyotrophic lateral sclerosis   RNA processing   alternative polyadenylation   neurodegeneration   biomarker  

Introduction and Academic Background Frontotemporal Dementia (FTD) and Amyotrophic Lateral Sclerosis (ALS) are two severe neurodegenerative diseases whose pathogenic mechanisms remain incompletely understood. In recent years, the RNA-binding protein TDP-43 (TAR DNA-binding protein 43) has been recognized as playing a central pathological role in bo...

TDP-43 Loss Induces Cryptic Polyadenylation in ALS/FTD

Medicine, Basic Medical Sciences, Neurology, Life Sciences, Cell Biology, Biochemistry, Genetics,
TDP-43   cryptic polyadenylation   ALS   FTD   RNA processing   neurodegeneration  

TDP-43 Loss Induces Cryptic Polyadenylation in ALS/FTD Background Introduction Amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) are two severe neurodegenerative diseases affecting hundreds of thousands of people worldwide. Extensive research has shown that the RNA-binding protein TDP-43 (TAR DNA-binding protein 43) displays abn...

Embryonic Motor Neuron Programming Factors Reactivate Immature Gene Expression and Suppress ALS Pathologies in Postnatal Motor Neurons

Medicine, Basic Medical Sciences, Neurology, Life Sciences, Cell Biology, Biochemistry, Bioengineering, Genetics,
motor neuron   ALS   transcription factors   gene expression   neurodegeneration   reprogramming   gene therapy  

1. Academic Background and Research Motivation Degenerative diseases of motor neurons, such as Amyotrophic Lateral Sclerosis (ALS), have long been a key research area in neuroscience. ALS is characterized by adult onset, with progressive degeneration of motor neurons leading to paralysis and death. In diseases such as ALS, aging is considered a maj...

Muscle-derived miR-126 regulates TDP-43 axonal local synthesis and NMJ integrity in ALS models

Medicine, Basic Medical Sciences, Neurology, Life Sciences, Cell Biology, Biochemistry, Bioengineering, Genetics,
muscle-derived miR-126   TDP-43 axonal synthesis   neuromuscular junction   ALS models   neurodegeneration   local protein synthesis  

Muscle-derived miR-126 Regulates Axonal Local Synthesis of TDP-43 to Maintain Neuromuscular Junction Integrity in ALS Models — Review of a Nature Neuroscience Article I. Academic Background and Research Motivation Amyotrophic lateral sclerosis (ALS) is a fatal adult-onset motor neuron disease, primarily characterized by neuromuscular junction (NMJ)...

Transmission of Peripheral Blood α-Synuclein Fibrils Exacerbates Synucleinopathy and Neurodegeneration in Parkinson’s Disease by Endothelial LAG3 Endocytosis

Medicine, Neurology, Life Sciences, Cell Biology, Immunology,
α-synuclein fibrils   Parkinson’s disease   LAG3 endocytosis   peripheral blood transmission   neurodegeneration  

Transmission of Peripheral Blood α-Synuclein Fibrils Exacerbates Synucleinopathy and Neurodegeneration in Parkinson’s Disease via Endothelial LAG3 Endocytosis Academic Background Parkinson’s disease (PD) is an age-related neurodegenerative disorder characterized by the abnormal formation and transmission of α-synuclein (α-syn). Recent studies have ...

Amyloid-Associated Hyperconnectivity Drives Tau Spread Across Connected Brain Regions in Alzheimer’s Disease

Medicine, Neurology,
Alzheimer's disease   tau spread   amyloid   neuronal hyperconnectivity   brain regions   neurodegeneration   functional connectivity  

Amyloid-Associated Hyperconnectivity Drives Tau Spread in Alzheimer’s Disease Academic Background Alzheimer’s Disease (AD) is a common neurodegenerative disorder characterized by the accumulation of amyloid-beta (Aβ) plaques and the abnormal aggregation and spread of tau protein in the brain. The traditional “amyloid cascade hypothesis” posits that...

GYS1 Antisense Therapy Prevents Disease-Driving Aggregates and Epileptiform Discharges in a Lafora Disease Mouse Model

Medicine, Basic Medical Sciences, Neurology, Life Sciences, Cell Biology, Biochemistry, Immunology,
GYS1   antisense oligonucleotide   epilepsy   Lafora disease   glycogen synthesis   neuroinflammation   neurodegeneration  

GYS1 Antisense Therapy Inhibits Pathogenic Aggregates and Epileptiform Discharges in a Mouse Model of Lafora Disease Background and Objectives Lafora disease (LD) is a devastating autosomal recessive genetic disorder characterized by epilepsy and rapidly progressive dementia in adolescence. The disease primarily involves mutations in the EPM2A or E...