Drug screening for ALS using patient-specific induced pluripotent stem cells

N Egawa, S Kitaoka, K Tsukita, M Naitoh… - Science translational …, 2012 - science.org
Amyotrophic lateral sclerosis (ALS) is a late-onset, fatal disorder in which the motor neurons
degenerate. The discovery of new drugs for treating ALS has been hampered by a lack of …

The Src/c-Abl pathway is a potential therapeutic target in amyotrophic lateral sclerosis

K Imamura, Y Izumi, A Watanabe, K Tsukita… - Science Translational …, 2017 - science.org
Amyotrophic lateral sclerosis (ALS), a fatal disease causing progressive loss of motor
neurons, still has no effective treatment. We developed a phenotypic screen to repurpose …

[HTML][HTML] Linear ubiquitination is involved in the pathogenesis of optineurin-associated amyotrophic lateral sclerosis

S Nakazawa, D Oikawa, R Ishii, T Ayaki… - Nature …, 2016 - nature.com
Optineurin (OPTN) mutations cause neurodegenerative diseases, including amyotrophic
lateral sclerosis (ALS) and glaucoma. Although the ALS-associated E478G mutation in the …

[HTML][HTML] Aberrant assembly of RNA recognition motif 1 links to pathogenic conversion of TAR DNA-binding protein of 43 kDa (TDP-43)

A Shodai, T Morimura, A Ido, T Uchida, T Ayaki… - Journal of Biological …, 2013 - ASBMB
Aggregation of TAR DNA-binding protein of 43 kDa (TDP-43) is a pathological signature of
amyotrophic lateral sclerosis (ALS). Although accumulating evidence suggests the …

α-Synuclein BAC transgenic mice exhibit RBD-like behaviour and hyposmia: a prodromal Parkinson's disease model

T Taguchi, M Ikuno, M Hondo, LK Parajuli, K Taguchi… - Brain, 2020 - academic.oup.com
Parkinson's disease is one of the most common movement disorders and is characterized by
dopaminergic cell loss and the accumulation of pathological α-synuclein, but its precise …

[HTML][HTML] Failure of DNA double-strand break repair by tau mediates Alzheimer's disease pathology in vitro

M Asada-Utsugi, K Uemura, T Ayaki… - Communications …, 2022 - nature.com
DNA double-strand break (DSB) is the most severe form of DNA damage and accumulates
with age, in which cytoskeletal proteins are polymerized to repair DSB in dividing cells …

[PDF][PDF] Pathological endogenous α-synuclein accumulation in oligodendrocyte precursor cells potentially induces inclusions in multiple system atrophy

S Kaji, T Maki, H Kinoshita, N Uemura, T Ayaki… - Stem cell reports, 2018 - cell.com
Glial cytoplasmic inclusions (GCIs), commonly observed as α-synuclein (α-syn)-positive
aggregates within oligodendrocytes, are the pathological hallmark of multiple system …

[HTML][HTML] Elimination of TDP-43 inclusions linked to amyotrophic lateral sclerosis by a misfolding-specific intrabody with dual proteolytic signals

Y Tamaki, A Shodai, T Morimura, R Hikiami… - Scientific reports, 2018 - nature.com
Aggregation of TAR DNA-binding protein of 43 kDa (TDP-43) is implicated in the
pathogenesis of sporadic and certain familial forms of amyotrophic lateral sclerosis (ALS) …

[HTML][HTML] Immunoreactivity of valosin-containing protein in sporadic amyotrophic lateral sclerosis and in a case of its novel mutant

T Ayaki, H Ito, H Fukushima, T Inoue, T Kondo… - Acta neuropathologica …, 2014 - Springer
Background Mutations in the valosin-containing protein (VCP) gene were first found to
cause inclusion-body myopathy with early-onset Paget disease and frontotemporal …

[HTML][HTML] DOPAnization of tyrosine in α-synuclein by tyrosine hydroxylase leads to the formation of oligomers

M Jin, S Matsumoto, T Ayaki, H Yamakado… - Nature …, 2022 - nature.com
Parkinson's disease is a progressive neurodegenerative disorder characterized by the
preferential loss of tyrosine hydroxylase (TH)-expressing dopaminergic neurons in the …