title: The microcephaly gene aspm is involved in brain development in zebrafish.
abstract: MCPH is a neurodevelopmental disorder characterized by a global reduction in cerebral cortical volume. Homozygous mutation of the MCPH5 gene, also known as ASPM, is the most common cause of the MCPH phenotype. To elucidate the roles of ASPM during embryonic development, the zebrafish aspm was identified, which is specifically expressed in proliferating cells in the CNS. Morpholino-mediated knock-down of aspm resulted in a significant reduction in head size. Furthermore, aspm-deficient embryos exhibited a mitotic arrest during early development. These findings suggest that the reduction in brain size in MCPH might be caused by lack of aspm function in the mitotic cell cycle and demonstrate that the zebrafish can provide a model system for congenital diseases of the human nervous system.
authors.affiliation: Department of Biology and GRAST, Chungnam National University, Daejeon 305-764, Republic of Korea.
authors.forename: Hyun-Taek
authors.initials: HT
authors.lastname: Kim
authors.forename: Mi-Sun
authors.initials: MS
authors.lastname: Lee
authors.forename: Jung-Hwa
authors.initials: JH
authors.lastname: Choi
authors.forename: Ju-Yeon
authors.initials: JY
authors.lastname: Jung
authors.forename: Dae-Gwon
authors.initials: DG
authors.lastname: Ahn
authors.forename: Sang-Yeob
authors.initials: SY
authors.lastname: Yeo
authors.forename: Dong-Kug
authors.initials: DK
authors.lastname: Choi
authors.forename: Cheol-Hee
authors.initials: CH
authors.lastname: Kim
mesh_terms: D000595:Amino Acid Sequence; D000818:Animals; D001921:Brain / Q000196:embryology*; D002453:Cell Cycle; D018797:Cell Cycle Proteins / Q000235:genetics*; D055785:Gene Knockdown Techniques; D006801:Humans; D008831:Microcephaly / Q000235:genetics*; D008938:Mitosis / Q000235:genetics; D008969:Molecular Sequence Data; D009929:Organ Size / Q000235:genetics; D015027:Zebrafish / Q000196:embryology* / Q000235:genetics; D029961:Zebrafish Proteins / Q000235:genetics*
chemical_list: C560754:ASPM protein, zebrafish; D018797:Cell Cycle Proteins; D029961:Zebrafish Proteins