Hideaki Matsui
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View article: Behavioral and molecular insights into anxiety in ube3a and fmr1 zebrafish models of autism spectrum disorders
Behavioral and molecular insights into anxiety in ube3a and fmr1 zebrafish models of autism spectrum disorders Open
Angelman syndrome and Fragile X syndrome are neurodevelopmental disorders (NDDs) caused by mutations in the UBE3A and FMR1 genes, respectively. However, they share common features such as cognitive and motor deficits, anxiety, and impaired…
View article: Mutation of ube3a causes developmental abnormalities and autism-like molecular and behavioral alterations in zebrafish
Mutation of ube3a causes developmental abnormalities and autism-like molecular and behavioral alterations in zebrafish Open
Mutations in the UBE3A gene are responsible for neurodevelopmental disorders (NDDs), including Angelman syndrome (AS), which is characterized by developmental delays, impaired motor coordination, and cognitive disabilities. In recent years…
View article: Exploring α-Syn’s Functions Through Ablation Models: Physiological and Pathological Implications
Exploring α-Syn’s Functions Through Ablation Models: Physiological and Pathological Implications Open
View article: Three decades of neuroscience research using animal models of ADHD and ASD: a bibliometric analysis
Three decades of neuroscience research using animal models of ADHD and ASD: a bibliometric analysis Open
Introduction Attention-deficit/hyperactivity disorder (ADHD) and autism spectrum disorders (ASD) are two increasingly prevalent neurodevelopmental disorders (NDDs), often accompanied by significant daily-life challenges. Animal models play…
View article: Lipofuscin autofluorescence confounds intracellular amyloid β detection in the aged mouse brain
Lipofuscin autofluorescence confounds intracellular amyloid β detection in the aged mouse brain Open
Intracellular amyloid β (Aβ) accumulation is a contentious feature of Alzheimer's disease (AD), increasingly reported in young adults and aged animal models of AD. However, autofluorescent lipofuscin granules which consist of a mixture of …
View article: A robust protocol for proteomic profiling of secreted proteins in conditioned culture medium
A robust protocol for proteomic profiling of secreted proteins in conditioned culture medium Open
Reliable secretome analysis is crucial for understanding cellular communication and developing therapeutic strategies. However, conventional protein quantification methods, such as the bicinchoninic acid (BCA) assay, can overestimate prote…
View article: Homozygous slc25a20 zebrafish mutant reveals insights into carnitine-acylcarnitine translocase deficiency pathogenesis
Homozygous slc25a20 zebrafish mutant reveals insights into carnitine-acylcarnitine translocase deficiency pathogenesis Open
View article: Single housing of juveniles accelerates early-stage growth but extends adult lifespan in African turquoise killifish
Single housing of juveniles accelerates early-stage growth but extends adult lifespan in African turquoise killifish Open
Within the same species, individuals exhibiting faster growth tend to have shorter lifespans, even if their fast growth arises from early-life pharmacological interventions. However, in vertebrates, the impact of the early-life environment…
View article: Current trends in basic research on Parkinson’s disease: from mitochondria, lysosome to α-synuclein
Current trends in basic research on Parkinson’s disease: from mitochondria, lysosome to α-synuclein Open
View article: Extracellular disposal of nuclear waste by APP: a protective mechanism impaired in Alzheimer’s disease
Extracellular disposal of nuclear waste by APP: a protective mechanism impaired in Alzheimer’s disease Open
Although the amyloid beta (Aβ) hypothesis 1 has long been central to Alzheimer’s disease (AD) research, effective therapeutic strategies remain elusive 2,3 . Here we re-evaluate the functions of amyloid precursor protein (APP) and reveal i…
View article: The transcription factor NF-YA is crucial for neural progenitor maintenance during brain development
The transcription factor NF-YA is crucial for neural progenitor maintenance during brain development Open
In contrast to stage-specific transcription factors, the role of ubiquitous transcription factors in neuronal development remains a matter of scrutiny. Here, we demonstrated that a ubiquitous factor NF-Y is essential for neural progenitor …
View article: Phosphorylation of α-synuclein at T64 results in distinct oligomers and exerts toxicity in models of Parkinson’s disease
Phosphorylation of α-synuclein at T64 results in distinct oligomers and exerts toxicity in models of Parkinson’s disease Open
α-Synuclein accumulates in Lewy bodies, and this accumulation is a pathological hallmark of Parkinson’s disease (PD). Previous studies have indicated a causal role of α-synuclein in the pathogenesis of PD. However, the molecular and cellul…
View article: Functional regionalization of the differentiating cerebellar Purkinje cell population occurs in an activity-dependent manner
Functional regionalization of the differentiating cerebellar Purkinje cell population occurs in an activity-dependent manner Open
Introduction The cerebellum is organized into functional regions each dedicated to process different motor or sensory inputs for controlling different locomotor behaviors. This functional regionalization is prominent in the evolutionary co…
View article: Fish Models for Exploring Mitochondrial Dysfunction Affecting Neurodegenerative Disorders
Fish Models for Exploring Mitochondrial Dysfunction Affecting Neurodegenerative Disorders Open
Neurodegenerative disorders are characterized by the progressive loss of neuronal structure or function, resulting in memory loss and movement disorders. Although the detailed pathogenic mechanism has not been elucidated, it is thought to …
View article: Glutamatergic pathways in the brains of turtles: A comparative perspective among reptiles, birds, and mammals
Glutamatergic pathways in the brains of turtles: A comparative perspective among reptiles, birds, and mammals Open
Glutamate acts as the main excitatory neurotransmitter in the brain and plays a vital role in physiological and pathological neuronal functions. In mammals, glutamate can cause detrimental excitotoxic effects under anoxic conditions. In co…
View article: The Utility of Small Fishes for the Genetic Study of Human Age-Related Disorders
The Utility of Small Fishes for the Genetic Study of Human Age-Related Disorders Open
Animal models have been used to model human diseases, and among them, small fishes have been highlighted for their usefulness in various ways, such as the low cost of maintenance, ease of genetic modification, small size for easy handling,…
View article: Rapid reverse genetics systems for Nothobranchius furzeri, a suitable model organism to study vertebrate aging
Rapid reverse genetics systems for Nothobranchius furzeri, a suitable model organism to study vertebrate aging Open
View article: Modelling Autism Spectrum Disorder (ASD) and Attention-Deficit/Hyperactivity Disorder (ADHD) Using Mice and Zebrafish
Modelling Autism Spectrum Disorder (ASD) and Attention-Deficit/Hyperactivity Disorder (ADHD) Using Mice and Zebrafish Open
Autism spectrum disorders (ASD) and attention-deficit/hyperactivity disorder (ADHD) are two debilitating neurodevelopmental disorders. The former is associated with social impairments whereas the latter is associated with inattentiveness, …
View article: Rapid reverse genetics systems for N. furzeri, a suitable model organism to study vertebrate aging
Rapid reverse genetics systems for N. furzeri, a suitable model organism to study vertebrate aging Open
The African turquoise killifish ( N. furzeri ) is a useful model organism for studying aging, age-related diseases, and embryonic diapause. CRISPR/Cas9-mediated gene knockout and Tol2 transposon-mediated transgenesis in N. furzeri have bee…
View article: Evaluation of Ectopic Mitochondrial DNA in HeLa Cells
Evaluation of Ectopic Mitochondrial DNA in HeLa Cells Open
The presence of ectopic DNA in the cytoplasm induces inflammation and cell death. It has been widely reported that leakage of nuclear DNA into the cytoplasm can mainly be sensed by cyclic GMP-AMP synthase (cGAS). We recently reported that …
View article: Zebrafish, Medaka and Turquoise Killifish for Understanding Human Neurodegenerative/Neurodevelopmental Disorders
Zebrafish, Medaka and Turquoise Killifish for Understanding Human Neurodegenerative/Neurodevelopmental Disorders Open
In recent years, small fishes such as zebrafish and medaka have been widely recognized as model animals. They have high homology in genetics and tissue structure with humans and unique features that mammalian model animals do not have, suc…
View article: Cellular response against cytosolic leakage of mitochondrial DNA: insights into the pathology of Parkinson’s disease
Cellular response against cytosolic leakage of mitochondrial DNA: insights into the pathology of Parkinson’s disease Open
Mitochondria are important organelles for cellular metabolism and homeostasis, and their abnormalities are implicated in various diseases. Since mitochondria originate from protobacterium, their components are easily recognized by pathogen…
View article: Cytosolic dsDNA of mitochondrial origin induces cytotoxicity and neurodegeneration in cellular and zebrafish models of Parkinson’s disease
Cytosolic dsDNA of mitochondrial origin induces cytotoxicity and neurodegeneration in cellular and zebrafish models of Parkinson’s disease Open
Mitochondrial dysfunction and lysosomal dysfunction have been implicated in Parkinson’s disease (PD), but the links between these dysfunctions in PD pathogenesis are still largely unknown. Here we report that cytosolic dsDNA of mitochondri…
View article: Degeneration of dopaminergic neurons and impaired intracellular trafficking in Atp13a2 deficient zebrafish
Degeneration of dopaminergic neurons and impaired intracellular trafficking in Atp13a2 deficient zebrafish Open
View article: F-ready: A support device that allows disabled people to play the guitar
F-ready: A support device that allows disabled people to play the guitar Open
Although many people with disabilities have a strong desire to play a musical instrument, there are a few support devices that allow them to play it. We propose four considerations that have been identified for the development of such a su…
View article: Age- and α-Synuclein-Dependent Degeneration of Dopamine and Noradrenaline Neurons in the Annual Killifish Nothobranchius furzeri
Age- and α-Synuclein-Dependent Degeneration of Dopamine and Noradrenaline Neurons in the Annual Killifish Nothobranchius furzeri Open
Parkinson's disease (PD) is a neurodegenerative disease characterized by α-synuclein-positive inclusion bodies and loss of neurons, including dopaminergic neurons. Difficulty in replicating PD phenotypes using animal models partly limits t…
View article: An optimized method for counting dopaminergic neurons in zebrafish
An optimized method for counting dopaminergic neurons in zebrafish Open
In recent years, considerable effort has been devoted to the development of a fish model for Parkinson's disease (PD) to examine the pathological mechanisms of neurodegeneration. To effectively evaluate PD pathology, the ability to accurat…
View article: Dopamine system, cerebellum, and nucleus ruber in fish and mammals
Dopamine system, cerebellum, and nucleus ruber in fish and mammals Open
Small teleost fish including zebrafish and medaka have been used as animal models for research because of their small body size, vast amounts of eggs produced, their rapid development, low husbandry costs, and transparency during embryogen…
View article: Analyzing Synaptic Modulation of <em>Drosophila melanogaster</em> Photoreceptors after Exposure to Prolonged Light
Analyzing Synaptic Modulation of <em>Drosophila melanogaster</em> Photoreceptors after Exposure to Prolonged Light Open
The nervous system has the remarkable ability to adapt and respond to various stimuli. This neural adjustment is largely achieved through plasticity at the synaptic level. The Active Zone (AZ) is the region at the presynaptic membrane that…
View article: Impairment of the autophagy-lysosome pathway in <i>GBA</i><sup>-/-</sup> medaka.
Impairment of the autophagy-lysosome pathway in <i>GBA</i><sup>-/-</sup> medaka. Open
(A) Ubiquitin, p62, and Cathepsin D immunohistochemistry in the neuronal layer of the optic tectum at 3 months. Almost all of the cells in these figures are neurons. Ubiquitin-positive and p62-positive aggregates were observed in the perik…