研究者詳細

顔写真

ラジヤ　ノラズリーン　ビンテイ　ラジヤ　アハマド
Raja Norazireen Binti Raja Ahmad
Raja Norazireen Binti Raja Ahmad
所属
加齢医学研究所　附属環境ストレス老化研究センター　核酸修飾・損傷応答研究分野
職名
助教
学位

  • Doctor of Philosophy, Medical Science （Kumamoto University）

e-Rad 研究者番号
50999761

経歴 7

  • 2024年4月 ～ 継続中
    Institute of Development, Aging and Cancer, Tohoku University

  • 2022年4月 ～ 2024年3月
    Institute of Development, Aging and Cancer, Tohoku University

  • 2017年9月 ～ 2022年3月
    Graduate School of Medical Sciences, Kumamoto University

  • 2015年4月 ～ 2017年4月
    Cell Therapy Centre, Universiti Kebangsaan Malaysia Medical Centre

  • 2013年7月 ～ 2015年3月
    Virtuallux Sdn Bhd

  • 2012年1月 ～ 2013年6月
    Inno Bio Diagnostics Sdn Bhd

  • 2008年7月 ～ 2011年12月
    Inno Bio Diagnostics Sdn Bhd

︎全件表示 ︎最初の5件までを表示

学歴 3

  • Graduate School of Medical Sciences, Kumamoto University

    2018年4月 ～ 2022年3月

  • Graduate School of Science and Engineering for Education, University of Toyama

    2006年4月 ～ 2008年3月

  • Faculty of Engineering, University of Toyama

    2002年4月 ～ 2006年3月

論文 4

  1. Pathological mutations promote proteolysis of mitochondrial tRNA-specific 2-thiouridylase 1 (MTU1) via mitochondrial caseinolytic peptidase (CLPP)

    Raja Norazireen Raja Ahmad, Long-Teng Zhang, Rikuri Morita, Haruna Tani, Yong Wu, Takeshi Chujo, Akiko Ogawa, Ryuhei Harada, Yasuteru Shigeta, Kazuhito Tomizawa, Fan-Yan Wei

    Nucleic Acids Research　52　(3)　1341-1358　2023年12月19日

    出版者・発行元： Oxford University Press (OUP)

    DOI： 10.1093/nar/gkad1197 　

    ISSN：0305-1048

    eISSN：1362-4962

    詳細を見る 詳細を閉じる

    Abstract MTU1 controls intramitochondrial protein synthesis by catalyzing the 2-thiouridine modification of mitochondrial transfer RNAs (mt-tRNAs). Missense mutations in the MTU1 gene are associated with life-threatening reversible infantile hepatic failure. However, the molecular pathogenesis is not well understood. Here, we investigated 17 mutations associated with this disease, and our results showed that most disease-related mutations are partial loss-of-function mutations, with three mutations being particularly severe. Mutant MTU1 is rapidly degraded by mitochondrial caseinolytic peptidase (CLPP) through a direct interaction with its chaperone protein CLPX. Notably, knockdown of CLPP significantly increased mutant MTU1 protein expression and mt-tRNA 2-thiolation, suggesting that accelerated proteolysis of mutant MTU1 plays a role in disease pathogenesis. In addition, molecular dynamics simulations demonstrated that disease-associated mutations may lead to abnormal intermolecular interactions, thereby impairing MTU1 enzyme activity. Finally, clinical data analysis underscores a significant correlation between patient prognosis and residual 2-thiolation levels, which is partially consistent with the AlphaMissense predictions. These findings provide a comprehensive understanding of MTU1-related diseases, offering prospects for modification-based diagnostics and novel therapeutic strategies centered on targeting CLPP.

  2. Regulation of mitochondrial iron homeostasis by sideroflexin 2

    Ei Ei Mon, Fan-Yan Wei, Raja Norazireen Raja Ahmad, Takahiro Yamamoto, Toshiro Moroishi, Kazuhito Tomizawa

    The Journal of Physiological Sciences　69　(2)　359-373　2018年12月20日

    出版者・発行元： Springer Science and Business Media LLC

    DOI： 10.1007/s12576-018-0652-2 　

    ISSN：1880-6546

    eISSN：1880-6562

  3. Establishment of Stable and Secretable TATκ-GFP Recombinant Protein: A Preliminary Report of Promoter Methylation in 293T Cell Line

    Zariyantey Abd Hamid, Fazlina Nordin, Raja Norazireen Raja Ahmad, Balqis Mat Rashid, Ubashini Vijakumaran

    Sains Malaysiana　47　(10)　2473-2480　2018年10月31日

    出版者・発行元： Penerbit Universiti Kebangsaan Malaysia (UKM Press)

    DOI： 10.17576/jsm-2018-4710-24 　

    ISSN：0126-6039

  4. Transactivator protein: An alternative for delivery of recombinant proteins for safer reprogramming of induced Pluripotent Stem Cell

    Fazlina Nordin, Raja Norazireen Raja Ahmad, Farzin Farzaneh

    Virus Research　235　106-114　2017年5月

    出版者・発行元： Elsevier BV

    DOI： 10.1016/j.virusres.2017.04.007 　

    ISSN：0168-1702

講演・口頭発表等 1

  1. Supersulfide in RNA modification and metabolic diseases 招待有り

    Raja Norazireen Raja Ahmad

    1st International G-ReXs Conference, April 2024

共同研究・競争的資金等の研究課題 1

  1. ミトコンドリアタンパク質品質管理による翻訳制御機構の解明

    RAJA・NORAZIREEN・BINTI RAJA・AHMAD

    提供機関：Japan Society for the Promotion of Science

    制度名：Grants-in-Aid for Scientific Research

    研究種目：Grant-in-Aid for Research Activity Start-up

    研究機関：Tohoku University

    2024年7月31日 ～ 2026年3月31日