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 …
RNA-related metabolism. Hyper-phosphorylated and ubiquitinated TDP-43 deposits act as …
[HTML][HTML] The role of mitochondria in amyotrophic lateral sclerosis
Mitochondria are unique organelles that are essential for a variety of cellular processes
including energy metabolism, calcium homeostasis, lipid biosynthesis, and apoptosis …
including energy metabolism, calcium homeostasis, lipid biosynthesis, and apoptosis …
Converging mechanisms in ALS and FTD: disrupted RNA and protein homeostasis
Breakthrough discoveries identifying common genetic causes for amyotrophic lateral
sclerosis (ALS) and frontotemporal dementia (FTD) have transformed our view of these …
sclerosis (ALS) and frontotemporal dementia (FTD) have transformed our view of these …
RNA in unexpected places: long non-coding RNA functions in diverse cellular contexts
The increased application of transcriptome-wide profiling approaches has led to an
explosion in the number of documented long non-coding RNAs (lncRNAs). While these new …
explosion in the number of documented long non-coding RNAs (lncRNAs). While these new …
Postmortem cortex samples identify distinct molecular subtypes of ALS: retrotransposon activation, oxidative stress, and activated glia
OH Tam, NV Rozhkov, R Shaw, D Kim, I Hubbard… - Cell reports, 2019 - cell.com
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease characterized by
the progressive loss of motor neurons. While several pathogenic mutations have been …
the progressive loss of motor neurons. While several pathogenic mutations have been …
Physiological functions and pathobiology of TDP‐43 and FUS/TLS proteins
The multiple roles played by RNA binding proteins in neurodegeneration have become
apparent following the discovery of TAR DNA binding protein 43 kDa (TDP‐43) and fused in …
apparent following the discovery of TAR DNA binding protein 43 kDa (TDP‐43) and fused in …
Stress granules as crucibles of ALS pathogenesis
Amyotrophic lateral sclerosis (ALS) is a fatal human neurodegenerative disease affecting
primarily motor neurons. Two RNA-binding proteins, TDP-43 and FUS, aggregate in the …
primarily motor neurons. Two RNA-binding proteins, TDP-43 and FUS, aggregate in the …
[HTML][HTML] Axonal transport of TDP-43 mRNA granules is impaired by ALS-causing mutations
NH Alami, RB Smith, MA Carrasco, LA Williams… - Neuron, 2014 - cell.com
The RNA-binding protein TDP-43 regulates RNA metabolism at multiple levels, including
transcription, RNA splicing, and mRNA stability. TDP-43 is a major component of the …
transcription, RNA splicing, and mRNA stability. TDP-43 is a major component of the …
Disruption of RNA metabolism in neurological diseases and emerging therapeutic interventions
RNA binding proteins are critical to the maintenance of the transcriptome via controlled
regulation of RNA processing and transport. Alterations of these proteins impact multiple …
regulation of RNA processing and transport. Alterations of these proteins impact multiple …
Divergent roles of ALS-linked proteins FUS/TLS and TDP-43 intersect in processing long pre-mRNAs
FUS/TLS (fused in sarcoma/translocated in liposarcoma) and TDP-43 are integrally involved
in amyotrophic lateral sclerosis (ALS) and frontotemporal dementia. We found that FUS/TLS …
in amyotrophic lateral sclerosis (ALS) and frontotemporal dementia. We found that FUS/TLS …