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The present study focused on zebrafish (Danio rerio) belonging to Cypriniformes as an experimental animal model with neu1 gene deficiency. The results revealed that the zebrafish Neu1 desialyzed both \u03b12\u20133 and \u03b12\u20136 sialic acid linkages from oligosaccharides and glycoproteins at pH 4.5, and it is highly conserved with other fish species and mammalian Neu1. Furthermore, Neu1-knockout zebrafish (Neu1-KO) was established through CRISPR\/Cas9 genome editing. Neu1-KO fish exhibited slight abnormal embryogenesis with the accumulation of pleural effusion; however, no embryonic lethality was observed. Although Neu1-KO fish were able to be maintained as homozygous, they showed smaller body length and weight than the wild-type (WT) fish, and muscle atrophy and curvature of the vertebra were observed in adult Neu1-KO fish (8 months). The expression patterns of myod and myog transcription factors regulating muscle differentiation varied between Neu1-KO and WT fish embryo. Expression of lysosomal-related genes, including ctsa, lamp1a, and tfeb were up-regulated in adult Neu1-KO muscle as compared with WT. Furthermore, the expression pattern of genes involved in bone remodeling (runx2a, runx2b, and mmp9) was decreased in Neu1-KO fish. These phenotypes were quite similar to those of Neu1-KO mice and human sialidosis patients, indicating the effectiveness of the established Neu1-KO zebrafish for the study of vertebrate Neu1 sialidase.<\/jats:p>","DOI":"10.1042\/bcj20200348","type":"journal-article","created":{"date-parts":[[2020,7,20]],"date-time":"2020-07-20T11:36:07Z","timestamp":1595244967000},"page":"2841-2857","update-policy":"https:\/\/doi.org\/10.1042\/crossmark_policy","source":"Crossref","is-referenced-by-count":17,"title":["Establishment and characterization of Neu1-knockout zebrafish and its abnormal clinical phenotypes"],"prefix":"10.1042","volume":"477","author":[{"given":"Keiji","family":"Okada","sequence":"first","affiliation":[{"name":"Faculty of Fisheries, Kagoshima University, Kagoshima, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ryo","family":"Takase","sequence":"additional","affiliation":[{"name":"Faculty of Fisheries, Kagoshima University, Kagoshima, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yurie","family":"Hamaoka","sequence":"additional","affiliation":[{"name":"Faculty of Fisheries, Kagoshima University, Kagoshima, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Akinobu","family":"Honda","sequence":"additional","affiliation":[{"name":"The United Graduate School of Agricultural Sciences, Kagoshima University, Kagoshima, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Asami","family":"Ikeda","sequence":"additional","affiliation":[{"name":"The United Graduate School of Agricultural Sciences, Kagoshima University, Kagoshima, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yoichiro","family":"Hokazono","sequence":"additional","affiliation":[{"name":"Faculty of Fisheries, Kagoshima University, Kagoshima, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yutaro","family":"Maeda","sequence":"additional","affiliation":[{"name":"Faculty of Fisheries, Kagoshima University, Kagoshima, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Oki","family":"Hayasaka","sequence":"additional","affiliation":[{"name":"The United Graduate School of Agricultural Sciences, Kagoshima University, Kagoshima, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tomonari","family":"Kotani","sequence":"additional","affiliation":[{"name":"Faculty of Fisheries, Kagoshima University, Kagoshima, Japan"},{"name":"The United Graduate School of Agricultural Sciences, Kagoshima University, Kagoshima, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Masaharu","family":"Komatsu","sequence":"additional","affiliation":[{"name":"Faculty of Fisheries, Kagoshima University, Kagoshima, Japan"},{"name":"The United Graduate School of Agricultural Sciences, Kagoshima University, Kagoshima, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0523-838X","authenticated-orcid":false,"given":"Kazuhiro","family":"Shiozaki","sequence":"additional","affiliation":[{"name":"Faculty of Fisheries, Kagoshima University, Kagoshima, Japan"},{"name":"The United Graduate School of Agricultural Sciences, Kagoshima University, Kagoshima, Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"288","published-online":{"date-parts":[[2020,8,12]]},"reference":[{"key":"2021112218111454700_BCJ-2020-0348C1","doi-asserted-by":"publisher","first-page":"e44193","DOI":"10.1371\/journal.pone.0044193","article-title":"New insights on the sialidase protein family revealed by a phylogenetic analysis in Metazoa","volume":"7","year":"2012","journal-title":"PLoS ONE"},{"key":"2021112218111454700_BCJ-2020-0348C2","doi-asserted-by":"publisher","first-page":"567","DOI":"10.1007\/s10719-012-9394-1","article-title":"Sialidase significance for cancer progression","volume":"29","year":"2012","journal-title":"Glycoconj. 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