Molecular mechanisms of TDP-43 misfolding and pathology in amyotrophic lateral sclerosis

A Prasad, V Bharathi, V Sivalingam… - Frontiers in molecular …, 2019 - frontiersin.org
TAR DNA binding protein 43 (TDP-43) is a versatile RNA/DNA binding protein involved in
RNA-related metabolism. Hyper-phosphorylated and ubiquitinated TDP-43 deposits act as …

Epigenetic regulation in neurodegenerative diseases

A Berson, R Nativio, SL Berger, NM Bonini - Trends in neurosciences, 2018 - cell.com
Mechanisms of epigenetic regulation, including DNA methylation, chromatin remodeling,
and histone post-translational modifications, are involved in multiple aspects of neuronal …

[HTML][HTML] p53 is a central regulator driving neurodegeneration caused by C9orf72 poly (PR)

M Maor-Nof, Z Shipony, R Lopez-Gonzalez… - Cell, 2021 - cell.com
The most common genetic cause of amyotrophic lateral sclerosis (ALS) and frontotemporal
dementia (FTD) is a GGGGCC repeat expansion in the C9orf72 gene. We developed a …

CRISPR–Cas9 screens in human cells and primary neurons identify modifiers of C9ORF72 dipeptide-repeat-protein toxicity

NJ Kramer, MS Haney, DW Morgens, A Jovičić… - Nature …, 2018 - nature.com
Hexanucleotide-repeat expansions in the C9ORF72 gene are the most common cause of
amyotrophic lateral sclerosis and frontotemporal dementia (c9ALS/FTD). The nucleotide …

Maternal immune activation in mice disrupts proteostasis in the fetal brain

BT Kalish, E Kim, B Finander, EE Duffy, H Kim… - Nature …, 2021 - nature.com
Maternal infection and inflammation during pregnancy are associated with
neurodevelopmental disorders in offspring, but little is understood about the molecular …

Small molecule modulators of chromatin remodeling: from neurodevelopment to neurodegeneration

D Jiang, T Li, C Guo, TS Tang, H Liu - Cell & Bioscience, 2023 - Springer
The dynamic changes in chromatin conformation alter the organization and structure of the
genome and further regulate gene transcription. Basically, the chromatin structure is …

Exploring options for proximity-dependent biotinylation experiments: comparative analysis of labeling enzymes and affinity purification resins

KJ Schreiber, E Kadijk, JY Youn - Journal of Proteome Research, 2024 - ACS Publications
Proximity-dependent biotinylation (PDB) techniques provide information about the molecular
neighborhood of a protein of interest, yielding insights into its function and localization. Here …

[HTML][HTML] Emerging therapies and novel targets for TDP-43 proteinopathy in ALS/FTD

LR Hayes, P Kalab - Neurotherapeutics, 2022 - Elsevier
Nuclear clearance and cytoplasmic mislocalization of the essential RNA binding protein,
TDP-43, is a pathologic hallmark of amyotrophic lateral sclerosis, frontotemporal dementia …

Molecular hallmarks of ageing in amyotrophic lateral sclerosis

CJ Jagaraj, S Shadfar, SA Kashani… - Cellular and Molecular …, 2024 - Springer
Amyotrophic lateral sclerosis (ALS) is a fatal, severely debilitating and rapidly progressing
disorder affecting motor neurons in the brain, brainstem, and spinal cord. Unfortunately …

Epigenetics in amyotrophic lateral sclerosis: a role for histone post-translational modifications in neurodegenerative disease

SA Bennett, R Tanaz, SN Cobos, MP Torrente - Translational research, 2019 - Elsevier
Amyotrophic lateral sclerosis (ALS) is the third most common adult onset neurodegenerative
disorder worldwide. It is generally characterized by progressive paralysis starting at the …