APP mouse models for Alzheimer's disease preclinical studies

H Sasaguri, P Nilsson, S Hashimoto, K Nagata… - The EMBO …, 2017 - embopress.org
Animal models of human diseases that accurately recapitulate clinical pathology are
indispensable for understanding molecular mechanisms and advancing preclinical studies. The …

[HTML][HTML] Recent advances in the modeling of Alzheimer's disease

H Sasaguri, S Hashimoto, N Watamura… - Frontiers in …, 2022 - frontiersin.org
Since 1995, more than 100 transgenic (Tg) mouse models of Alzheimer’s disease (AD)
have been generated in which mutant amyloid precursor protein (APP) or APP/presenilin 1 (PS1) …

C9ORF72 repeat expansions in mice cause TDP-43 pathology, neuronal loss, and behavioral deficits

J Chew, TF Gendron, M Prudencio, H Sasaguri… - Science, 2015 - science.org
The major genetic cause of frontotemporal dementia and amyotrophic lateral sclerosis is a G
4 C 2 repeat expansion in C9ORF72. Efforts to combat neurodegeneration associated with “…

[HTML][HTML] Aggregation-prone c9FTD/ALS poly (GA) RAN-translated proteins cause neurotoxicity by inducing ER stress

…, TF Gendron, KF Bieniek, WL Lin, H Sasaguri… - Acta …, 2014 - Springer
The occurrence of repeat-associated non-ATG (RAN) translation, an atypical form of translation
of expanded repeats that results in the synthesis of homopolymeric expansion proteins, …

C9ORF72 poly(GA) aggregates sequester and impair HR23 and nucleocytoplasmic transport proteins

YJ Zhang, TF Gendron, JC Grima, H Sasaguri… - Nature …, 2016 - nature.com
Neuronal inclusions of poly(GA), a protein unconventionally translated from G 4 C 2 repeat
expansions in C9ORF72, are abundant in patients with frontotemporal dementia (FTD) and …

[HTML][HTML] The extreme N-terminus of TDP-43 mediates the cytoplasmic aggregation of TDP-43 and associated toxicity in vivo

H Sasaguri, J Chew, YF Xu, TF Gendron, A Garrett… - Brain research, 2016 - Elsevier
Inclusions of Tar DNA- binding protein 43 (TDP-43) are a pathological hallmark of
amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration with TDP-43-positive …

The dual functions of the extreme N-terminus of TDP-43 in regulating its biological activity and inclusion formation

…, J Hubbard, C Stetler, H Sasaguri… - Human molecular …, 2013 - academic.oup.com
TAR DNA-binding protein-43 (TDP-43) is the principal component of ubiquitinated inclusions
in amyotrophic lateral sclerosis (ALS) and the most common pathological subtype of …

[HTML][HTML] Humanization of the entire murine Mapt gene provides a murine model of pathological human tau propagation

T Saito, N Mihira, Y Matsuba, H Sasaguri… - Journal of Biological …, 2019 - ASBMB
In cortical regions of brains from individuals with preclinical or clinical Alzheimer's disease (AD),
extracellular β-amyloid (Aβ) deposition precedes the aggregation of pathological …

Non-human primate model of amyotrophic lateral sclerosis with cytoplasmic mislocalization of TDP-43

A Uchida, H Sasaguri, N Kimura, M Tajiri, T Ohkubo… - Brain, 2012 - academic.oup.com
Amyotrophic lateral sclerosis is a fatal neurodegenerative disease characterized by
progressive motoneuron loss. Redistribution of transactive response deoxyribonucleic acid-binding …

[HTML][HTML] Depletion of vitamin E increases amyloid β accumulation by decreasing its clearances from brain and blood in a mouse model of Alzheimer disease

…, N Iwata, K Jishage, H Yamada, H Sasaguri… - Journal of Biological …, 2009 - ASBMB
Increased oxidative damage is a prominent and early feature in Alzheimer disease. We
previously crossed Alzheimer disease transgenic (APPsw) model mice with α-tocopherol transfer …