Gains or losses: molecular mechanisms of TDP43-mediated neurodegeneration
RNA-binding proteins, and in particular TAR DNA-binding protein 43 (TDP43), are central to
the pathogenesis of motor neuron diseases and related neurodegenerative disorders …
the pathogenesis of motor neuron diseases and related neurodegenerative disorders …
The genetics and neuropathology of amyotrophic lateral sclerosis
A Al-Chalabi, A Jones, C Troakes, A King… - Acta …, 2012 - Springer
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease of motor neurons
leading to death from respiratory failure within about 3 years of symptom onset. A family …
leading to death from respiratory failure within about 3 years of symptom onset. A family …
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 …
Mutant induced pluripotent stem cell lines recapitulate aspects of TDP-43 proteinopathies and reveal cell-specific vulnerability
Transactive response DNA-binding (TDP-43) protein is the dominant disease protein in
amyotrophic lateral sclerosis (ALS) and a subgroup of frontotemporal lobar degeneration …
amyotrophic lateral sclerosis (ALS) and a subgroup of frontotemporal lobar degeneration …
EPHA4 is a disease modifier of amyotrophic lateral sclerosis in animal models and in humans
A Van Hoecke, L Schoonaert, R Lemmens… - Nature medicine, 2012 - nature.com
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease affecting motor
neurons. Disease onset and progression are variable, with survival ranging from months to …
neurons. Disease onset and progression are variable, with survival ranging from months to …
[HTML][HTML] FUS-SMN protein interactions link the motor neuron diseases ALS and SMA
T Yamazaki, S Chen, Y Yu, B Yan, TC Haertlein… - Cell reports, 2012 - cell.com
Mutations in the RNA binding protein FUS cause amyotrophic lateral sclerosis (ALS), a fatal
adult motor neuron disease. Decreased expression of SMN causes the fatal childhood motor …
adult motor neuron disease. Decreased expression of SMN causes the fatal childhood motor …
TMEM106B, the risk gene for frontotemporal dementia, is regulated by the microRNA-132/212 cluster and affects progranulin pathways
AS Chen-Plotkin, TL Unger, MD Gallagher… - Journal of …, 2012 - Soc Neuroscience
Frontotemporal lobar degeneration with TDP-43 inclusions (FTLD-TDP) is a fatal
neurodegenerative disease with no available treatments. Mutations in the progranulin gene …
neurodegenerative disease with no available treatments. Mutations in the progranulin gene …
The complex molecular biology of amyotrophic lateral sclerosis (ALS)
RL Redler, NV Dokholyan - Progress in molecular biology and translational …, 2012 - Elsevier
Amyotrophic lateral sclerosis (ALS) is an adult-onset neurodegenerative disorder that
causes selective death of motor neurons followed by paralysis and death. A subset of ALS …
causes selective death of motor neurons followed by paralysis and death. A subset of ALS …
Misregulated RNA processing in amyotrophic lateral sclerosis
Amyotrophic lateral sclerosis (ALS) research is undergoing an era of unprecedented
discoveries with the identification of new genes as major genetic causes of this disease …
discoveries with the identification of new genes as major genetic causes of this disease …
Frontotemporal dementia: implications for understanding Alzheimer disease
M Goedert, B Ghetti… - Cold Spring …, 2012 - perspectivesinmedicine.cshlp.org
Frontotemporal dementia (FTD) comprises a group of behavioral, language, and movement
disorders. On the basis of the nature of the characteristic protein inclusions, frontotemporal …
disorders. On the basis of the nature of the characteristic protein inclusions, frontotemporal …