Brain Research Institute

Carla Koehler, Ph.D.

Email Address:
koehlerc@chem.ucla.edu
Work Email Address:
koehler@chem.ucla.edu

Laboratory Address:
Young Hall 4043

Mailing Address:
Department of Chemistry and Biochemistry UCLA
Mail Code: 156905
607 Charles Young Drive East
Los Angeles, CA 90095
UNITED STATES

Work Address:
Young Hall 4041A

Fax Number:
(310) 206-4038
Lab Number:
1 (310) 825-8427
Work Phone Number:
1 (310) 794-4834

Department / Division Affiliations

Lab Director, Koehler Lab

Professor, Chemistry and Biochemistry, Biochemistry, Molecular Biology

Member, ACCESS Program: Depts. of Biochemistry & Molecular Biology, JCCC Cancer and Stem Cell Biology Program Area, Brain Research Institute, Jonsson Comprehensive Cancer Center, Molecular Biology Institute

Faculty, Molecular Biology IDP

Researcher, Biochemistry, Chemical Biology

Bio:

Professor Koehler has been a faculty member in the Department of Chemistry and Biochemistry since 1999. She came to UCLA after doing post-doctoral research in the laboratory of Dr Gottfried Schatz at the Biozentrum, Basel, Switzerland. Her research characterized a new protein import pathway in the mitochondrion and linked a defect in protein import with the inherited disease deafness-dystonia syndrome. Dr. Koehler completed her graduate studies at Iowa State University, characterizing the inheritance of mitochondrial DNA during her M.S. studies and studying dimorphism in Saccharomyces cerevisiae. Dr. Koehler is a former Damon Runyon Scholar and current Established Investigator of the American Heart Association. Dr. Koehler is an Associate Editor for Current Genetics. She has been a member of the Molecular Biology Institute, Jonsson Cancer Center, and the Brain Research Institute since 1999. Her current research focuses on developing vertebrate models for mitochondrial diseases as well as using yeast for mechanistic studies on mitochondrial biogenesis.

Publications:

Lu, G., H. Sun, P. Korge, C. M. Koehler, J. N. Weiss, and Y. Wang Functional characterization of a mitochondrial Ser/Thr protein phosphatase in cell death regulation. Methods Enzymol.. 2009; 457: 255-273.
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Chacinska A., C. M. Koehler, D. Milenkovic, T. Lithgow, and N. Pfanner Importing mitochondrial proteins: machineries and mechanisms. Cell. 2009; 138: 628-644.
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Tienson, H. L., D. V. Dabir, S. E. Neal, R. Loo, S. A. Hasson, P. Boontheung, S.-K. Kim, J. A. Loo, and C. M. Koehler Reconstitution of the Mia40-Erv1 oxidative folding pathway for the small Tim proteins. Mol. Biol. Cell. 2009; 20: 3481-3490.
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Koehler, C. M. and H. L. Tienson Redox regulation of protein folding in the mitochondrial intermembrane space. Biochim Biophys Acta. 2009; 1793: 139-145.
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Gebert, N., A. Chacinska, K. Wagner, B. Guiard, C. M. Koehler, P. Rehling, N. Pfanner, and N. Wiedemann Assembly of the three small Tim proteins precedes docking to the mitochondrial carrier translocase. EMBO Rep.. 2008; 9: 548-554.
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Claypool, S. M., Y. Oktay, P. Boontheung, J. A. Loo, and C. M. Koehler Cardiolipin defines the interactome of the major ADP/ATP carrier protein of the mitochondrial inner membrane. J. Cell Biol.. 2008; 182: 937-950.
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Walsh, S. and C. M. Koehler Gazing at translocation in the mitochondrion. Cell. 2008; 134: 382-383.
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Beverly, K. N., M, R. Sawaya, E. Schmid, and C. M. Koehler The Tim8-Tim13 complex has multiple substrate binding sites and binds cooperatively to Tim23. J. Mol. Biol.. 2008; 382: 1144-1156.
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Claypool, S. M., P. Boontheung, J. M. McCaffery, J. A. Loo, and C. M. Koehler The cardiolipin transacylase, Tafazzin, associates with two distinct respiratory components providing insight into Barth syndrome. Mol. Biol. Cell.. 2008; 19: 5143-5155.
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Lu G., Ren S., Korge P., Choi J., Dong Y., Weiss J., Koehler C., Chen J.N., Wang Y. A novel mitochondrial matrix serine/threonine protein phosphatase regulates the mitochondria permeability transition pore and is essential for cellular survival and development. Genes Dev. 2007; 21: 784-796.

Dabir, D. V., E. P. Leverich, S.-K. Kim, F. D. Tsai, M. Hirasawa, D. B. Knaff, and C. M. Koehler A role for cytochrome c and cytochrome c peroxidase in electron shuttling from Erv1. EMBO J. 2007; 26: 4801-4811.

Sediva, A., C. I. E. Smith, A. C. Asplund, J. Hadac, A. Janda, J. Zeman, H. Hansikova; L. Dvorakova, L. Mrazova, S. Velbri, C. Koehler, K. Roesch, K. E. Sullivan, T. Futatani, H. Ochs Contiguous X-chromosome deletion syndrome encompassing the BTK, TIMM8A, TAF7L and DRP2 genes. J. Clin. Immunol. 2007; 27: 640-646.

Chen, H.-W., C. M. Koehler, and M. A. Teitell Human polynucleotide phosphorylase: Location matters. Trends Cell Biol. 2007; 17: 600-608.

Stuart, R.A. and C.M. Koehler In Vitro Analysis of Yeast Mitochondrial Protein Import. In Morgan, K. (ed.) Current Protocols in Cell Biology 2007; 11.19.1-11.19.20.

French S.W., Dawson D.W., Chen H.W., Rainey R.N., Sievers S.A., Balatoni C.E., Wong L., Troke J.J., Nguyen M.T., Koehler C.M., Teitell M.A. The TCL1 oncoprotein binds the RNase PH domains of the PNPase exoribonuclease without affecting its RNA degrading activity. Cancer Letts. 2007; 248: 198-210.

Oktay, Y., R.N.Rainey, and C.M. Koehler The function of TIM22 in the insertion of inner membrane proteins in mitochondria. Dalbey, R.E., Koehler, C.M., and Tamanoi, F. (eds.) The Enzymes: Molecular Machines Involved in Protein Transport across Membrane 2007; 25: 367-386.

Hwang, D. K., S. M. Claypool, H. D. Tienson, D. Leuenberger, C. M. Koehler Tim54p mediates assembly of the i-AAA protease Yme1p. J. Cell Biol. 2007; 178: 1161-75.

Rainey R.N., Glavin J.D., Chen H.W., French S.W., Teitell M.A., Koehler C.M. A new function in translocation for the mitochondrial i-AAA protease Yme1: import of polynucleotide phosphorylase into the intermembrane space.. Mol. Cell. Biol. 2006; 26(22): 8488-8497.

Chen, H.W., Rainey, R.N., Balatoni C.E., Dawson D.W., Troke J.J., Wasiak S., Hong J.S., McBride H.M., Koehler C.M., Teitell M.A., French S.W. Mammalian polynucleotide phosphorylase is an intermembrane space RNase that maintains mitochondrial homeostasis. Mol. Cell. Biol. 2006; 26(22): 8475-8487.

Claypool, S.M., McCaffery, J.M., Koehler, C.M. Mitochondrial mislocalization and altered assembly of a cluster of Barth syndrome mutant tafazzins. J. Cell Biol. 2006; 184: 379-390.

Koehler, C.M., Beverly, K.N., Leverich, E.P. Redox Pathways of the Mitochondrion. Antioxid. Redox. Signal. 2006; 8: 813-822.

Likic VA, Perry A, Hulett J,Derby M, Traven A, Waller RF, Keeling PJ, Koehler CM, Curran SP, Gooley PR and Lithgow T Patterns that define the four domains conserved in known and novel isoforms of the protein import receptor Tom20. J. Mol. Biol.. 2005; 347: 81-93.

Koehler CM, Hwang DK Protein translocation across membranes.. Encyclopedia of Molecular Cell Biology and Molecular Medicine 2005; 11: 287-308.

Curran SP and Koehler CM Mitochondrial biogenesis. Protein import into and across the inner membrane. Topics in Current Genetics: Mitochondrial Function and Biogenesis 2004; 8: 59-80.

Koehler CM New developments in mitochondrial assembly.. Annual review of cell and developmental biology. . 2004; 20: 309-35.

Leuenberger D, Curran SP and Koehler CM Structure, bioenergetics, and biogenesis of mitochondria. The Biogenesis of Cellular Organelles 2004; 138-163.

Roesch K, Hynds PH, Varga R, Tranebjaerg L and Koehler CM The calcium-binding apartate/glutamate carriers, citrin and aralar1, are new substrates for the DDP1/TIMM8a-TIMM13 complex. Hum. Mol. Gen.. 2004; 13: 2101-2111.

Koehler CM Import and assembly. Genetics of Mitochondrial Diseases 2003; 47-68.

Dyall SD, Lester DC, Schneider RE, Delgadillo-Correa MG, Plumper E, Martinez A, Koehler CM, Johnson PJ Trichomonas vaginalis Hmp35, a putative pore-forming hydrogenosomal membrane protein, can form a complex in yeast mitochondria. J. Biol. Chem.. 2003; 278: 30548-30461.

Roesch K, Tranejaerg L, Koehler CM How do defects in mitochondrial protein import lead to deafness. 19th Danavox Symposium: Genetics and the function of the auditory system. 2002; 19: 139-154.

Roesch K, Curran SP, Tranebjaerg L and Koehler CM Human deafness dystonia syndrome is caused by a defect in asssembly of the DDP1/TIMM8a-TIMM13 complex.. Hum. Mol. Gent.. 2002; 11: 477-486.

Murphy MP, Leuenberger D, Curran SP, Oppliger W and Koehler CM The essential function of the small tim proteins in the tim22 import pathway does not depend on formation of the soluble 70-kilodalton complex.. Mol. Cell. Biol.. 2001; 21: 6132-6138.

Renold A, Koehler CM and Murphy MP Mitochondrial import of the long and short isoforms of human uncoupling protein 3.. FEBS Lett.. 2000; 465: 135-140.

Koehler CM Protein translocation pathways of the mitochondrion.. FEBS Lett.. 2000; 476(1-2): 27-31.

Dyall SM, Koehler CM, Delgadillo MG, Bradley PJ, Plumper E, Leuenberger D, Turck CW and Johnson PJ The presence of mitochondria carrier in the hydrogenosomal membranes: conservation of membrane targeting pathways indicated a progenitor organelle gave rise to hydrogenosomes and mitochondria.. Mol. Cell. Biol.. 2000; 20: 2488-2497.

Koehler CM, Murphy MP, Bally NA, Oppliger W, Dolfini L, Junne T and Or E Tim18p, a novel subunit of the inner membrane complex that mediates protein import into the yeast mitochondrial inner membrane.. Mol. Cell. Biol.. 2000; 20: 1187-1193.

Koehler CM, Leuenberger D, Merchant S, Renold A, Junne T and Schatz G Human deafness dystonia syndrome is a mitochondrial disease.. Proc. Natl. Acad. Sci. (USA). 1999; 96: 2141-2146.

Komiya T, Rospert S, Koehler CM, Looser R, Schatz G, and Mihara K Interaction of mitochondrial targeting signals with acidic receptor domains along the protein import pathway: Evidence for the "acid chain" hypothesis.. EMBO J 1998; 17: 3886-3898.

Koehler CM, Merchant S, Oppliger W, Schmid K, Jarosch E, Dolfini L, Junne T, Schatz G and Tokatlidis K Tim9p, an essential partner of Tim10p for the import of mitochondrial carriers.. EMBO J. 1998; 17: 6477-6486.

Koehler CM, Lindber GL, BrownDR, Beitz DC, Freeman AE, Mayfield JE and Myers AM Replacement of bovine mitochondrial DNA by a sequence variant within a single generation.. Genetics 1991; 129: 247-255.

Claypool SM, Koehler CM Hereditary spastic paraplegia: respiratory choke or unactivated substrate?. Cell. . 2005; 123(2): 183-5.

Curran SP, Leverich EP, Koehler CM, Larsen PL Defective mitochondrial protein translocation precludes normal Caenorhabditis elegans development.. The Journal of biological chemistry. . 2004; 279(52): 54655-62.

Curran SP, Leuenberger D, Leverich EP, Hwang DK, Beverly KN, Koehler CM The role of Hot13p and redox chemistry in the mitochondrial TIM22 import pathway.. The Journal of biological chemistry. . 2004; 279(42): 43744-51.

Koehler CM The small Tim proteins and the twin Cx3C motif.. Trends in biochemical sciences. . 2004; 29(1): 1-4.

van der Bliek AM, Koehler CM A mitochondrial rhomboid protease.. Developmental cell. . 2003; 4(6): 769-70.

Leuenberger D, Curran SP, Wong D, Koehler CM The role of Tim9p in the assembly of the TIM22 import complexes.. Traffic (Copenhagen, Denmark) . 2003; 4(3): 144-52.

Curran SP, Leuenberger D, Schmidt E, Koehler CM The role of the Tim8p-Tim13p complex in a conserved import pathway for mitochondrial polytopic inner membrane proteins.. The Journal of cell biology. . 2002; 158(6): 1017-27.

Curran SP, Leuenberger D, Oppliger W, Koehler CM The Tim9p-Tim10p complex binds to the transmembrane domains of the ADP/ATP carrier.. The EMBO journal. . 2002; 21(5): 942-53.

Patterson SD, Spahr CS, Daugas E, Susin SA, Irinopoulou T, Koehler C, Kroemer G Mass spectrometric identification of proteins released from mitochondria undergoing permeability transition.. Cell death and differentiation. . 2000; 7(2): 137-44.

Koehler CM, Merchant S, Schatz G How membrane proteins travel across the mitochondrial intermembrane space.. Trends in biochemical sciences. . 1999; 24(11): 428-32.

Leuenberger D, Bally NA, Schatz G, Koehler CM Different import pathways through the mitochondrial intermembrane space for inner membrane proteins.. The EMBO journal. . 1999; 18(17): 4816-22.

Koehler CM, Jarosch E, Tokatlidis K, Schmid K, Schweyen RJ, Schatz G Import of mitochondrial carriers mediated by essential proteins of the intermembrane space.. Science. . 1998; 279(5349): 369-73.

Koehler CM, Myers AM Serine-threonine protein kinase activity of Elm1p, a regulator of morphologic differentiation in Saccharomyces cerevisiae.. FEBS letters. . 1997; 408(1): 109-14.

Blacketer MJ, Koehler CM, Coats SG, Myers AM, Madaule P Regulation of dimorphism in Saccharomyces cerevisiae: involvement of the novel protein kinase homolog Elm1p and protein phosphatase 2A.. Molecular and cellular biology. . 1993; 13(9): 5567-81.