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Molecular Pathogenesis of Neurodegeneration: From Fish to Human

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A special issue of International Journal of Molecular Sciences (ISSN 1422-0067). This special issue belongs to the section "Molecular Neurobiology".

Deadline for manuscript submissions: closed (30 November 2021) | Viewed by 15140

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Special Issue Editor

Prof. Dr. Hideaki Matsui

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Guest Editor

Department of Neuroscience of Disease, Brain Research Institute, Niigata University, Niigata 951-8585, Japan
Interests: neurodegeneration; Parkinson's disease; aging; zebrafish; medaka; neuroscience; autism
Special Issues, Collections and Topics in MDPI journals

Special Issue Information

Dear Colleagues,

Neurodegeneration is the progressive loss of neurons. Neurodegenerative diseases include Alzheimer's disease, Parkinson’s disease, motor neuron disease, triplet repeat diseases, Prion disease and multiple sclerosis. The number of patients is increasing and increasing due to the elongation of human life span. Researchers have long studied the mechanisms of these diseases, but the mechanisms are still largely unknown. This Special Issue focuses on molecular mechanisms contributing to the understanding of neurodegenerative diseases using small fish, relatively new animal models in the life sciences. Small fish including zebrafish and medaka possess unique properties for use as model animals, and this Special Issue will show the potential of using small fish for unveiling the mechanisms of neurodegenerative diseases. Original manuscripts and reviews dealing with the molecular pathogenesis of neurodegeneration using small fish are very welcome from outstanding experts on the topic.

Prof. Dr. Hideaki Matsui
Guest Editor

Manuscript Submission Information

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Keywords

zebrafish
medaka
neurodegeneration
neurological disease

Published Papers (4 papers)

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Research

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19 pages, 1975 KiB

Open AccessArticle

Efficient Neuroprotective Rescue of Sacsin-Related Disease Phenotypes in Zebrafish

by Valentina Naef, Maria Marchese, Asahi Ogi, Gianluca Fichi, Daniele Galatolo, Rosario Licitra, Stefano Doccini, Tiziano Verri, Francesco Argenton, Federica Morani and Filippo M. Santorelli

Int. J. Mol. Sci. 2021, 22(16), 8401; https://doi.org/10.3390/ijms22168401 - 5 Aug 2021

Cited by 8 | Viewed by 2770

Abstract

Autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS) is a multisystem hereditary ataxia associated with mutations in SACS, which encodes sacsin, a protein of still only partially understood function. Although mouse models of ARSACS mimic largely the disease progression seen in humans, their use in the validation of effective therapies has not yet been proposed. Recently, the teleost Danio rerio has attracted increasing attention as a vertebrate model that allows rapid and economical screening, of candidate molecules, and thus combines the advantages of whole-organism phenotypic assays and in vitro high-throughput screening assays. Through CRISPR/Cas9-based mutagenesis, we generated and characterized a zebrafish sacs-null mutant line that replicates the main features of ARSACS. The sacs-null fish showed motor impairment, hindbrain atrophy, mitochondrial dysfunction, and reactive oxygen species accumulation. As proof of principle for using these mutant fish in high-throughput screening studies, we showed that both acetyl-DL-leucine and tauroursodeoxycholic acid improved locomotor and biochemical phenotypes in sacs^−/− larvae treated with these neuroprotective agents, by mediating significant rescue of the molecular functions altered by sacsin loss. Taken together, the evidence here reported shows the zebrafish to be a valuable model organism for the identification of novel molecular mechanisms and for efficient and rapid in vivo optimization and screening of potential therapeutic compounds. These findings may pave the way for new interventions targeting the earliest phases of Purkinje cell degeneration in ARSACS. Full article

(This article belongs to the Special Issue Molecular Pathogenesis of Neurodegeneration: From Fish to Human)

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14 pages, 2654 KiB

Open AccessArticle

Genetic Modeling of the Neurodegenerative Disease Spinocerebellar Ataxia Type 1 in Zebrafish

by Mohamed A. Elsaey, Kazuhiko Namikawa and Reinhard W. Köster

Int. J. Mol. Sci. 2021, 22(14), 7351; https://doi.org/10.3390/ijms22147351 - 8 Jul 2021

Cited by 8 | Viewed by 3086

Abstract

Dominant spinocerebellar ataxias (SCAs) are progredient neurodegenerative diseases commonly affecting the survival of Purkinje cells (PCs) in the human cerebellum. Spinocerebellar ataxia type 1 (SCA1) is caused by the mutated ataxin1 (Atx1) gene product, in which a polyglutamine stretch encoded by CAG repeats is extended in affected SCA1 patients. As a monogenetic disease with the Atx1-polyQ protein exerting a gain of function, SCA1 can be genetically modelled in animals by cell type-specific overexpression. We have established a transgenic PC-specific SCA1 model in zebrafish coexpressing the fluorescent reporter protein mScarlet together with either human wild type Atx1[30Q] as control or SCA1 patient-derived Atx1[82Q]. SCA1 zebrafish display an age-dependent PC degeneration starting at larval stages around six weeks postfertilization, which continuously progresses during further juvenile and young adult stages. Interestingly, PC degeneration is observed more severely in rostral than in caudal regions of the PC population. Although such a neuropathology resulted in no gross locomotor control deficits, SCA1-fish with advanced PC loss display a reduced exploratory behaviour. In vivo imaging in this SCA1 model may help to better understand such patterned PC death known from PC neurodegeneration diseases, to elucidate disease mechanisms and to provide access to neuroprotective compound characterization in vivo. Full article

(This article belongs to the Special Issue Molecular Pathogenesis of Neurodegeneration: From Fish to Human)

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Review

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17 pages, 1122 KiB

Open AccessReview

Zebrafish, Medaka and Turquoise Killifish for Understanding Human Neurodegenerative/Neurodevelopmental Disorders

by Kazuki Kodera and Hideaki Matsui

Int. J. Mol. Sci. 2022, 23(3), 1399; https://doi.org/10.3390/ijms23031399 - 26 Jan 2022

Cited by 15 | Viewed by 4108

Abstract

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, such as transparency of embryos and larvae, a small body size and ease of experiments, including genetic manipulation. Zebrafish and medaka have been used extensively in the field of neurology, especially to unveil the mechanisms of neurodegenerative diseases such as Parkinson’s and Alzheimer’s disease, and recently, these fishes have also been utilized to understand neurodevelopmental disorders such as autism spectrum disorder. The turquoise killifish has emerged as a new and unique model animal, especially for ageing research due to its unique life cycle, and this fish also seems to be useful for age-related neurological diseases. These small fishes are excellent animal models for the analysis of human neurological disorders and are expected to play increasing roles in this field. Here, we introduce various applications of these model fishes to improve our understanding of human neurological disorders. Full article

(This article belongs to the Special Issue Molecular Pathogenesis of Neurodegeneration: From Fish to Human)

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17 pages, 3970 KiB

Open AccessReview

Zebrafish and Medaka: Important Animal Models for Human Neurodegenerative Diseases

by Jing Wang and Hong Cao

Int. J. Mol. Sci. 2021, 22(19), 10766; https://doi.org/10.3390/ijms221910766 - 5 Oct 2021

Cited by 26 | Viewed by 4463

Abstract

Animal models of human neurodegenerative disease have been investigated for several decades. In recent years, zebrafish (Danio rerio) and medaka (Oryzias latipes) have become popular in pathogenic and therapeutic studies about human neurodegenerative diseases due to their small size, the optical clarity of embryos, their fast development, and their suitability to large-scale therapeutic screening. Following the emergence of a new generation of molecular biological technologies such as reverse and forward genetics, morpholino, transgenesis, and gene knockout, many human neurodegenerative disease models, such as Parkinson’s, Huntington’s, and Alzheimer’s, were constructed in zebrafish and medaka. These studies proved that zebrafish and medaka genes are functionally conserved in relation to their human homologues, so they exhibit similar neurodegenerative phenotypes to human beings. Therefore, fish are a suitable model for the investigation of pathologic mechanisms of neurodegenerative diseases and for the large-scale screening of drugs for potential therapy. In this review, we summarize the studies in modelling human neurodegenerative diseases in zebrafish and medaka in recent years. Full article

(This article belongs to the Special Issue Molecular Pathogenesis of Neurodegeneration: From Fish to Human)

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