ZFIN ID: ZDB-PERS-060808-12
Koehler, Carla
Email: koehlerc@chem.ucla.edu
URL: http://koehler.chem.ucla.edu/
Affiliation: Koehler Lab
Address: UCLA/Dept of Chemistry and Biochemistry 4043 Young Hall 607 Charles E Young Dr East Box 951569 Los Angeles, CA 90095-1569 USA
Country: United States
Phone: 310 794-4834

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.

Langenbacher, A.D., Shimizu, H., Hsu, W., Zhao, Y., Borges, A., Koehler, C., Chen, J.N. (2020) Mitochondrial Calcium Uniporter Deficiency in Zebrafish Causes Cardiomyopathy With Arrhythmia. Frontiers in Physiology. 11:617492
Yien, Y.Y., Shi, J., Chen, C., Cheung, J.T., Grillo, A.S., Shrestha, R., Li, L., Zhang, X., Kafina, M.D., Kingsley, P.D., King, M.J., Ablain, J., Li, H., Zon, L., Palis, J., Burke, M.D., Bauer, D.E., Orkin, S.H., Koehler, C.M., Phillips, J.D., Kaplan, J., Ward, D.M., Lodish, H.F., Paw, B.H. (2018) FAM210B is an erythropoietin target and regulates erythroid heme synthesis by controlling mitochondrial iron import and ferrochelatase activity. The Journal of biological chemistry. 293(51):19797-19811
Francois-Moutal, L., Jahanbakhsh, S., Nelson, A., Ray, D., Scott, D.D., Hennefarth, M., Moutal, A., Perez-Miller, S., Ambrose, A.J., Al-Shamari, A., Coursodon, P., Meechoovet, B., Reiman, R., Lyons, E., Beilstein, M., Chapman, E., Morris, Q.D., Van Keuren-Jensen, K., Hughes, T.R., Khanna, R., Koehler, C., Jen, J., Gokhale, V., Khanna, M. (2018) A Chemical Biology Approach to Model Pontocerebellar Hypoplasia Type 1B (PCH1B). ACS Chemical Biology. 13(10):3000-3010
Sangwan, S., Zhao, A., Adams, K.L., Jayson, C.K., Sawaya, M.R., Guenther, E.L., Pan, A.C., Ngo, J., Moore, D.M., Soriaga, A.B., Do, T.D., Goldschmidt, L., Nelson, R., Bowers, M.T., Koehler, C.M., Shaw, D.E., Novitch, B.G., Eisenberg, D.S. (2017) Atomic structure of a toxic, oligomeric segment of SOD1 linked to amyotrophic lateral sclerosis (ALS). Proceedings of the National Academy of Sciences of the United States of America. 114(33):8770-8775
Miyata, N., Tang, Z., Conti, M.A., Johnson, M.E., Douglas, C.J., Hasson, S.A., Damoiseaux, R., Chang, C.A., Koehler, C.M. (2017) Adaptation of a Genetic Screen Reveals an Inhibitor for Mitochondrial Protein Import Component Tim44. The Journal of biological chemistry. 292:5429-5442
Wan, J., Steffen, J., Yourshaw, M., Mamsa, H., Andersen, E., Rudnik-Schöneborn, S., Pope, K., Howell, K.B., McLean, C.A., Kornberg, A.J., Joseph, J., Lockhart, P.J., Zerres, K., Ryan, M.M., Nelson, S.F., Koehler, C.M., Jen, J.C. (2016) Loss of function of SLC25A46 causes lethal congenital pontocerebellar hypoplasia. Brain : a journal of neurology. 139(11):2877-2890
Miyata, N., Steffen, J., Johnson, M.E., Fargue, S., Danpure, C.J., Koehler, C.M. (2014) Pharmacologic rescue of an enzyme-trafficking defect in primary hyperoxaluria 1. Proceedings of the National Academy of Sciences of the United States of America. 111:14406-11
Yien, Y. Y., Robledo, R. F., Schultz, I. J., Takahashi-Makise, N., Gwynn, B., Bauer, D. E., Bass, A., Yi, G., Li, L., Hildick-Smith, G. J., Cooney, J. D., Pierce, E. L., Mohler, K., Dailey, T. A., Miyata, N., Kingsley, P. D., Garone, C., Hattangadi, S. M., Huang, H., Chen, W., Keenan, E. M., Shah, D. I., Schlaeger, T. M., DiMauro, S., Orkin, S. H., Cantor, A. B., Palis, J., Koehler, C. M., Lodish, H. F., Kaplan, J., Ward, D. M., Dailey, H. A., Phillips, J. D., Peters, L. L., Paw, B. H. (2014) TMEM14C is required for erythroid mitochondrial heme metabolism. The Journal of Clinical Investigation. 124(10):4294-4304
Hildick-Smith, G.J., Cooney, J.D., Garone, C., Kremer, L.S., Haack, T.B., Thon, J.N., Miyata, N., Lieber, D.S., Calvo, S.E., Akman, H.O., Yien, Y.Y., Huston, N.C., Branco, D.S., Shah, D.I., Freedman, M.L., Koehler, C.M., Italiano, J.E., Merkenschlager, A., Beblo, S., Strom, T.M., Meitinger, T., Freisinger, P., Donati, M.A., Prokisch, H., Mootha, V.K., Dimauro, S., and Paw, B.H. (2013) Macrocytic Anemia and Mitochondriopathy Resulting from a Defect in Sideroflexin 4. American journal of human genetics. 93(5):906-14
Dabir, D.V., Hasson, S.A., Setoguchi, K., Johnson, M.E., Wongkongkathep, P., Douglas, C.J., Zimmerman, J., Damoiseaux, R., Teitell, M.A., and Koehler, C.M. (2013) A small molecule inhibitor of redox-regulated protein translocation into mitochondria. Developmental Cell. 25(1):81-92
Levesque, M.P., Krauss, J., Koehler, C., Boden, C., and Harris, M.P. (2013) New Tools for the Identification of Developmentally Regulated Enhancer Regions in Embryonic and Adult Zebrafish. Zebrafish. 10(1):21-9
Curado, S., Ober, E.A., Walsh, S., Cortes-Hernandez, P., Verkade, H., Koehler, C.M., and Stainier, D.Y. (2010) The mitochondrial import gene tomm22 is specifically required for hepatocyte survival and provides a liver regeneration model. Disease models & mechanisms. 3(7-8):486-495
Lu, G., Ren, S., Korge, P., Choi, J., Dong, Y., Weiss, J., Koehler, C., Chen, J.N, and Wang, Y. (2007) A novel mitochondrial matrix serine/threonine protein phosphatase regulates the mitochondria permeability transition pore and is essential for cellular survival and development. Genes & Development. 21(7):784-796