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First published online 21 June 2006
doi: 10.1242/dev.02457

Development 133, 3005-3013 (2006)
Published by The Company of Biologists 2006
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The murine homolog of SALL4, a causative gene in Okihiro syndrome, is essential for embryonic stem cell proliferation, and cooperates with Sall1 in anorectal, heart, brain and kidney development

Masayo Sakaki-Yumoto1,*, Chiyoko Kobayashi1,*, Akira Sato2, Sayoko Fujimura1, Yuko Matsumoto2, Minoru Takasato3, Tatsuhiko Kodama4, Hiroyuki Aburatani4, Makoto Asashima3, Nobuaki Yoshida5 and Ryuichi Nishinakamura1,2,6,*,{dagger}

1 Division of Integrative Cell Biology, Institute of Molecular Embryology and Genetics, Kumamoto University, Kumamoto 860-0811, Japan.
2 Division of Stem Cell Regulation, The Institute of Medical Science, The University of Tokyo, Tokyo 108-8639, Japan.
3 Graduate School of Sciences, The University of Tokyo, Tokyo 113-8654, Japan.
4 Research Center for Advanced Science and Technology, The University of Tokyo, Tokyo 153-8904, Japan.
5 Laboratory of Gene Expression and Regulation, The Institute of Medical Science, The University of Tokyo, Tokyo 108-8639, Japan.
6 PRESTO, JST, Saitama 332-0012, Japan.

{dagger} Author for correspondence (e-mail: ryuichi{at}kaiju.medic.kumamoto-u.ac.jp)

Accepted 24 May 2006

Mutations in SALL4, the human homolog of the Drosophila homeotic gene spalt (sal), cause the autosomal dominant disorder known as Okihiro syndrome. In this study, we show that a targeted null mutation in the mouse Sall4 gene leads to lethality during peri-implantation. Growth of the inner cell mass from the knockout blastocysts was reduced, and Sall4-null embryonic stem (ES) cells proliferated poorly with no aberrant differentiation. Furthermore, we demonstrated that anorectal and heart anomalies in Okihiro syndrome are caused by Sall4 haploinsufficiency and that Sall4/Sall1 heterozygotes exhibited an increased incidence of anorectal and heart anomalies, exencephaly and kidney agenesis. Sall4 and Sall1 formed heterodimers, and a truncated Sall1 caused mislocalization of Sall4 in the heterochromatin; thus, some symptoms of Townes-Brocks syndrome caused by SALL1 truncations could result from SALL4 inhibition.

Key words: Sall4, spalt, Embryonic stem cells, Okihiro syndrome, Townes-Brocks syndrome, Organogenesis, Mouse


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