个人简介

M.S. and Ph.D., Iowa State University Post-doctorate, Biozentrum, University of Basel

研究领域

Biochemistry/Systems Biology and Biological Regulation/Metabolism/Aging and Development/Chemical Biology

Our research encompasses two major areas: Understanding the mechanism of protein import into mitochondria and determining the process by which defects in mitochondrial protein translocation lead to disease. A basic question in cell biology is the mechanism by which a protein reaches its correct location within the cell. Of all the organelles in a mammalian cell, the mitochondrion is the most complex because two membranes must be crossed. In addition to the metabolic role, the mitochondria is a key player in many cellular processes including apoptosis, metal ion homeostasis and aging. My specific interests lie in mitochondrial biogenesis, particularly the mechanism by which proteins are imported into the mitochondrial inner membrane.

近期论文

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Pharmacologic rescue of an enzyme-trafficking defect in primary hyperoxaluria 1. Miyata N, Steffen J, Johnson ME, Fargue S, Danpure CJ, Koehler CM. Proc Natl Acad Sci U S A. 2014 Sep 18. pii: 201408401. [Epub ahead of print] PMID: 25237136 TMEM14C is required for erythroid mitochondrial heme metabolism. Yien YY, Robledo RF, Schultz IJ, Takahashi-Makise N, Gwynn B, Bauer DE, Dass A, Yi G, Li L, Hildick-Smith GJ, Cooney JD, Pierce EL, Mohler K, Dailey TA, Miyata N, Kingsley PD, Garone C, Hattangadi SM, Huang H, Chen W, Keenan EM, Shah DI, Schlaeger TM, DiMauro S, Orkin SH, Cantor AB, Palis J, Koehler CM, Lodish HF, Kaplan J, Ward DM, Dailey HA, Phillips JD, Peters LL, Paw BH. J Clin Invest. 2014 Oct 1;124(10):4294-304. doi: 10.1172/JCI76979. Epub 2014 Aug 26. PMID: 25157825 Mitochondria-targeted RNA import. Wang G, Shimada E, Nili M, Koehler CM, and Teitell MA. Methods Mol. Biol. 2014 In Press. The Taz1p transacylase is imported and sorted into the outer mitochondrial membrane via a membrane anchor domain. Herndon JD, Claypool SM, Koehler CM. Eukaryot Cell. 2013 Dec;12(12):1600-8. Macrocytic anemia and mitochondriopathy resulting from a defect in sideroflexin 4. Hildick-Smith GJ, Cooney JD, Garone C, Kremer LS, Haack TB, Thon JN, Miyata N, Lieber DS, Calvo SE, Akman HO, Yien YY, Huston NC, Branco DS, Shah DI, Freedman ML, Koehler CM, Italiano JE Jr, Merkenschlager A, Beblo S, Strom TM, Meitinger T, Freisinger P, Donati MA, Prokisch H, Mootha VK, DiMauro S, Paw BH. Am J Hum Genet. 2013 Nov 7;93(5):906-14. Meiosis I arrest abnormalities lead to severe oligozoospermia in meiosis 1 arresting protein (M1ap)-deficient mice. Arango NA, Li L, Dabir D, Nicolau F, Pieretti-Vanmarcke R, Koehler C, McCarrey JR, Lu N, Donahoe PK. Biol Reprod. 2013 Mar 28;88(3):76.