Kyushu University Academic Staff Educational and Research Activities Database
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Yuichi Matsushima Last modified date:2018.01.11

Assistant Professor / Department of Clinical Chemistry and Laboratory Medicine
Department of Basic Medicine
Faculty of Medical Sciences

Graduate School
Undergraduate School
Other Organization

Academic Degree
Ph. D.
Field of Specialization
Molecular Biology
Outline Activities
My research is centered around understanding the molecular mechanisms involved in the replication and expression of the mitochondrial DNA genome and their regulation.
Research Interests
  • Gene expression of mitochondrial DNA
    keyword : mitochondria
Academic Activities
1. Matsushima Y, Adan C, Garesse R, Kaguni LS, Mitochondria: Practical Protocols, Functional Analysis by Inducible RNA Interference in Drosophiola melanogaster, Humana Press, Methods in Molecular Biology, 372, 207-217, 2007.08.
1. Matsushima Y, Kaguni LS, Matrix proteases in mitochondrial DNA function. , Biochimica et Biophysica Acta., 2012.09.
1. Yuichi Matsushima*, Yuta Hirofuji, Masamune Aihara, Yue Song, Takeshi Uchiumi, Laurie S Kaguni, Dongchon Kang, Drosophila protease ClpXP specifically degrades DmLRPPRC1 controlling mitochondrial mRNA and translation, SCIENTIFIC REPORTS, 10.1038/s41598-017-08088-6, 7, 2017.08.
2. Chika Sakai, Yamaguchi, Seiji, Masayuki Sasaki, Yusaku Miyamoto, Yuichi Matsushima*, Yu-ichi Goto, ECHS1 Mutations Cause Combined Respiratory Chain Deficiency Resulting in Leigh Syndrome, HUMAN MUTATION, 10.1002/humu.22730, 36, 2, 232-239, 2015.02, 脂肪酸代謝酵素の異常は脂肪酸代謝異常症をていするが、本論文では脂肪酸代謝酵素の一つであるECHS1が、ミトコンドリア病の原因遺伝子であることを明らかにした。また、ECHS1の異常がが脂肪酸代謝異常を引き起こすだけでなく、ミトコンドリアの呼吸鎖活性の低下をしめすことを明らかにした。また、ECHS1変異が呼吸鎖複合体を阻害する代謝物の蓄積を引き起こしミトコンドリア病に至る可能性を示した。.
3. Matsushima Y, Goto Y, Kaguni LS, Mitochondrial Lon protease regulates mitochondrial DNA copy number and transcription by selective degradation of mitochondrial transcription factor A (TFAM). , Proc Natl Acad Sci USA, 107, 43, 18410-18415, 2010.10.
4. Matsushima Y, Farr CL, Fan L, Kaguni LS, Physiological and biochemical defects in carboxyl-terminal mutants of mitochondrial DNA helicase. , J Boil Chem, 283, 35, 23964-23971, 2008.08.
5. Matsushima Y, Kaguni LS, Differential phenotypes of active site and human autosomal dominant progressive external ophthalmoplegia mutations in drosophila mitochondrial DNA helicase expressed in Schneider cells. , J Boil Chem, 280, 17, 16815-16820, 2007.03.
6. Matsushima Y, Adan C, Garesse R, Kaguni LS, Drosophila mitochondrial transcription factor B1 modulates mitochondrial translation but not transcription or DNA copy number in Schneider cells. , J Boil Chem, 282, 13, 9436-9444, 2005.03.
7. Matsushima Y, Garesse R, Kaguni LS, Drosophila mitochondrial transcription factor B2 regulates mitochondrial DNA copy number and transcription in Schneider cells. , J Boil Chem, 279, 26, 26900-26905, 2004.06.
8. Matsushima Y, Matsumura K, Ishii S, Inagaki H, Suzuki T, Matsuda Y, Beck K, Kitagawa Y, Functional domains of chicken mitochondrial transcription factor a for the maintenance of mitochondrial DNA copy number in lymphoma cell line DT40. , J Boil Chem, 278, 33, 31149-31158, 2003.08.
Membership in Academic Society
  • Japan Society for Bioscience, Biotechnology, and Agrochemistry
  • Japanese Society of Mitochondrial Research and Medicine
  • The Japanese Biochemical Society
  • The Molecular Biology Society of Japan