A role for ATOX1 in 5q- syndrome

ATOX1 在 5q 综合征中的作用

• 批准号: 24659467
• 负责人: NAKAJIMA Hideaki
• 金额: $ 2.33万
• 依托单位: Keio University
• 依托单位国家: 日本
• 项目类别: Grant-in-Aid for Challenging Exploratory Research
• 财政年份: 2012
• 资助国家: 日本
• 起止时间: 2012-04-01 至 2014-03-31
• 项目状态: 已结题
• 来源: https://kaken.nii.ac.jp/en/grant/KAKENHI-PROJECT-24659467/
• 关键词: 5q-症候群; 骨髄異形成症候群; ATOX1; 造血幹細胞; 血球分化; 5q- 症候群

5q- syndrome is a subtype of myelodysplastic syndrome characterized by a deletion of chromosome 5q33. To elucidate molecular pathogenesis of 5q- syndrome, we focused on ATOX1, a cupper chaperone located in the common deleted region of 5q33. Frequencies of hematopoietic stem cells (HSCs), multipotent hematopoietic progenitors, and lineage-committed progenitors in the bone marrow were normal in ATOX1-/- mice, and ATOX1-/- HSCs maintained normal self-renewal and long-term repopulating capacities. These results suggest that ATOX1 is not essential for HSC function and hematopoietic differentiation, and that ATOX1 has little, if any, roles in 5q- syndrome.

5 q综合征是骨髓增生异常综合征的一种亚型，其特征在于染色体5 q33缺失。 为了阐明5 q综合征的分子发病机制，我们将重点放在ATOX 1上，ATOX 1是位于5 q33共同缺失区的铜分子伴侣。 在ATOX 1-/-小鼠中，骨髓中造血干细胞（HSC）、多能造血祖细胞和谱系定向祖细胞的频率是正常的，并且ATOX 1-/-HSC保持正常的自我更新和长期再生能力。 这些结果表明，ATOX 1不是必需的HSC功能和造血分化，ATOX 1有很少，如果有的话，在5 q综合征的作用。

项目成果

Disruption of Tet2 leads to enhanced self-renewal and competitive repopulating capacity of fetal liver hematopoietic stem cells

Tet2的破坏导致胎肝造血干细胞的自我更新和竞争性再生能力增强

• 发表时间: 2012
• 作者: Hiroyoshi Kunimoto;Yumi Fukuchi ;Masatoshi Sakurai;Ken Sadahira;Yasuo Ikeda;Shinichiro Okamoto;Hideaki Nakajima
• 通讯作者: Hideaki Nakajima

Direct reprogramming of terminally differentiated B cells into erythroid lineage

• DOI: 10.1016/j.febslet.2012.08.019
• 发表时间: 2012-10
• 期刊: FEBS Letters
• 影响因子: 3.5
• 作者: K. Sadahira;Y. Fukuchi;Hiroyoshi Kunimono;M. Sakurai;Y. Ikeda;S. Okamoto;H. Nakajima

The Genetic Landscape of FPD/AML Revealed CDC25C Mutation as a Driver that Promotes Malignant Transformation

FPD/AML 的遗传图谱揭示了 CDC25C 突变是促进恶性转化的驱动因素

• 发表时间: 2013
• 作者: Akihide Yoshimi;Takashi Toya;Masahiro Nakagawa;Masahito Kawazu;Yasuhito Nannya;Motoshi Ichikawa;Shunya Arai;Hironori Harada;Kensuke Usuki;Yasuhide Hayashi;Etsuro Ito;Keita Kirito;Hideaki Nakajima;Hiroyuki Mano;Mineo Kurokawa
• 通讯作者: Mineo Kurokawa

Chemo-Immunotherapy With Hyper-CVAD/MA and Rituximab For B-Cell Lymphomas Leads To Prolonged Immune Suppression Compared To Conventional Treatment With R-CHOP

与 R-CHOP 常规治疗相比，Hyper-CVAD/MA 和利妥昔单抗化疗免疫疗法治疗 B 细胞淋巴瘤可导致长期免疫抑制

• 发表时间: 2013
• 作者: Eri Matsuki;Taku Kikuchi;Norisato Hashimoto;Shunsuke Souma;Sumiko Kohashi;Takaaki Toyama;Masatoshi Sakurai;Daiki Karigane;Daichi Abe;Takayuki Shimizu;Kenji Yokoyama;Hideaki Nakajima;Shinichiro Okamoto
• 通讯作者: Shinichiro Okamoto

Impaired emergence of hematopoietic progenitors and defective megakaryocyte differentiation from FPD/AML-derived induced pluripotent stem cells

FPD/AML 衍生的诱导多能干细胞的造血祖细胞出现受损和巨核细胞分化缺陷

• 发表时间: 2013
• 作者: Masatoshi Sakurai;Hiroyoshi Kunimoto;Naohide Watanabe;Yumi Fukuchi;Shinsuke Yuasa;Keiichi Fukuda;Satoshi Yamazaki;Hiromitsu Nakauchi;Yasuhiro Ebihara;Koichiro Tsuji;Etsuro Ito;Yuka Harada;Hironori Harada;Shinichiro Okamoto;Hideaki Nakajima
• 通讯作者: Hideaki Nakajima