Motor neuron disease-associated loss of nuclear TDP-43 is linked to DNA double-strand break repair defects
J Mitra, EN Guerrero, PM Hegde… - Proceedings of the …, 2019 - National Acad Sciences
Genome damage and their defective repair have been etiologically linked to degenerating
neurons in many subtypes of amyotrophic lateral sclerosis (ALS) patients; however, the …
neurons in many subtypes of amyotrophic lateral sclerosis (ALS) patients; however, the …
Impaired NHEJ repair in amyotrophic lateral sclerosis is associated with TDP-43 mutations
Background Pathological forms of TAR DNA-binding protein 43 (TDP-43) are present in
motor neurons of almost all amyotrophic lateral sclerosis (ALS) patients, and mutations in …
motor neurons of almost all amyotrophic lateral sclerosis (ALS) patients, and mutations in …
TDP-43/FUS in motor neuron disease: Complexity and challenges
Amyotrophic lateral sclerosis (ALS), a common motor neuron disease affecting two per
100,000 people worldwide, encompasses at least five distinct pathological subtypes …
100,000 people worldwide, encompasses at least five distinct pathological subtypes …
Rethinking als: The fus about tdp-43
C Lagier-Tourenne, DW Cleveland - Cell, 2009 - cell.com
Mutations in TDP-43, a DNA/RNA-binding protein, cause an inherited form of the
neurodegenerative disease amyotrophic lateral sclerosis (ALS). Two recent studies …
neurodegenerative disease amyotrophic lateral sclerosis (ALS). Two recent studies …
TDP-43 mutations link Amyotrophic Lateral Sclerosis with R-loop homeostasis and R loop-mediated DNA damage
M Giannini, A Bayona-Feliu, D Sproviero… - PLoS …, 2020 - journals.plos.org
TDP-43 is a DNA and RNA binding protein involved in RNA processing and with structural
resemblance to heterogeneous ribonucleoproteins (hnRNPs), whose depletion sensitizes …
resemblance to heterogeneous ribonucleoproteins (hnRNPs), whose depletion sensitizes …
Amyotrophic lateral sclerosis-associated TDP-43 mutation Q331K prevents nuclear translocation of XRCC4-DNA ligase 4 complex and is linked to genome damage …
EN Guerrero, J Mitra, H Wang… - Human Molecular …, 2019 - academic.oup.com
Dominant mutations in the RNA/DNA-binding protein TDP-43 have been linked to
amyotrophic lateral sclerosis (ALS). Here, we screened genomic DNA extracted from spinal …
amyotrophic lateral sclerosis (ALS). Here, we screened genomic DNA extracted from spinal …
Understanding the role of TDP-43 and FUS/TLS in ALS and beyond
S Da Cruz, DW Cleveland - Current opinion in neurobiology, 2011 - Elsevier
Dominant mutations in two DNA/RNA binding proteins, TDP-43 and FUS/TLS, are causes of
inherited Amyotrophic Lateral Sclerosis (ALS). TDP-43 and FUS/TLS have striking structural …
inherited Amyotrophic Lateral Sclerosis (ALS). TDP-43 and FUS/TLS have striking structural …
DNA damage and repair deficiency in ALS/FTD-associated neurodegeneration: from molecular mechanisms to therapeutic implication
H Wang, M Kodavati, GW Britz… - Frontiers in Molecular …, 2021 - frontiersin.org
Emerging studies reveal that neurodegenerative disorders, including amyotrophic lateral
sclerosis (ALS) and frontotemporal dementia (FTD), are commonly linked to DNA damage …
sclerosis (ALS) and frontotemporal dementia (FTD), are commonly linked to DNA damage …
Partial loss of TDP-43 function causes phenotypes of amyotrophic lateral sclerosis
Amyotrophic lateral sclerosis (ALS) is a fatal neurological disease that causes motor neuron
degeneration, progressive motor dysfunction, paralysis, and death. Although multiple …
degeneration, progressive motor dysfunction, paralysis, and death. Although multiple …
[HTML][HTML] Dysregulation of the ALS-associated gene TDP-43 leads to neuronal death and degeneration in mice
Amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD) are
characterized by cytoplasmic protein aggregates in the brain and spinal cord that include …
characterized by cytoplasmic protein aggregates in the brain and spinal cord that include …