Carla Koehler*

Carla Marie Koehler

Professor, Chemistry and Biochemistry, University of California Los Angeles

UC Profile

Publications

  1. Han M, Bushong EA, Segawa M, Tiard A, Wong A, Brady MR, Momcilovic M, Wolf DM, Zhang R, Petcherski A, Madany M, Xu S, Lee JT, Poyurovsky MV, Olszewski K, Holloway T, Gomez A, John MS, Dubinett SM, Koehler CM, Shirihai OS, Stiles L, Lisberg A, Soatto S, Sadeghi S, Ellisman MH, Shackelford DB. Spatial mapping of mitochondrial networks and bioenergetics in lung cancer.. Nature, 2023.
  2. Liang WG, Wijaya J, Wei H, Noble AJ, Mancl JM, Mo S, Lee D, Lin King JV, Pan M, Liu C, Koehler CM, Zhao M, Potter CS, Carragher B, Li S, Tang WJ. Structural basis for the mechanisms of human presequence protease conformational switch and substrate recognition.. Nature communications, 2022.
  3. Zhang D, Dailey OR, Simon DJ, Roca-Datzer K, Jami-Alahmadi Y, Hennen MS, Wohlschlegel JA, Koehler CM, Dabir DV. Aim32 is a dual-localized 2Fe-2S mitochondrial protein that functions in redox quality control.. The Journal of biological chemistry, 2021.
  4. Langenbacher AD, Shimizu H, Hsu W, Zhao Y, Borges A, Koehler C, Chen JN. Mitochondrial Calcium Uniporter Deficiency in Zebrafish Causes Cardiomyopathy With Arrhythmia.. Frontiers in physiology, 2020.
  5. Momcilovic M, Shirihai O, Murphy MP, Koehler CM, Sadeghi S, Shackelford DB. Reply to: In vivo quantification of mitochondrial membrane potential.. Nature, 2020.
  6. Momcilovic M, Jones A, Bailey ST, Waldmann CM, Li R, Lee JT, Abdelhady G, Gomez A, Holloway T, Schmid E, Stout D, Fishbein MC, Stiles L, Dabir DV, Dubinett SM, Christofk H, Shirihai O, Koehler CM, Sadeghi S, Shackelford DB. Publisher Correction: In vivo imaging of mitochondrial membrane potential in non-small-cell lung cancer.. Nature, 2020.
  7. Momcilovic M, Jones A, Bailey ST, Waldmann CM, Li R, Lee JT, Abdelhady G, Gomez A, Holloway T, Schmid E, Stout D, Fishbein MC, Stiles L, Dabir DV, Dubinett SM, Christofk H, Shirihai O, Koehler CM, Sadeghi S, Shackelford DB. In vivo imaging of mitochondrial membrane potential in non-small-cell lung cancer.. Nature, 2019.
  8. Yien YY, Shi J, Chen C, Cheung JTM, Grillo AS, Shrestha R, Li L, Zhang X, Kafina MD, Kingsley PD, King MJ, Ablain J, Li H, Zon LI, Palis J, Burke MD, Bauer DE, Orkin SH, Koehler CM, Phillips JD, Kaplan J, Ward DM, Lodish HF, Paw BH. FAM210B is an erythropoietin target and regulates erythroid heme synthesis by controlling mitochondrial iron import and ferrochelatase activity.. The Journal of biological chemistry, 2018.
  9. François-Moutal L, Jahanbakhsh S, Nelson ADL, Ray D, Scott DD, Hennefarth MR, Moutal A, Perez-Miller S, Ambrose AJ, Al-Shamari A, Coursodon P, Meechoovet B, Reiman R, Lyons E, Beilstein M, Chapman E, Morris QD, Van Keuren-Jensen K, Hughes TR, Khanna R, Koehler C, Jen J, Gokhale V, Khanna M. A Chemical Biology Approach to Model Pontocerebellar Hypoplasia Type 1B (PCH1B).. ACS chemical biology, 2018.
  10. Shimada E, Ahsan FM, Nili M, Huang D, Atamdede S, TeSlaa T, Case D, Yu X, Gregory BD, Perrin BJ, Koehler CM, Teitell MA. PNPase knockout results in mtDNA loss and an altered metabolic gene expression program.. PloS one, 2018.
  11. Steffen J, Koehler CM. ER-mitochondria contacts: Actin dynamics at the ER control mitochondrial fission via calcium release.. The Journal of cell biology, 2017.
  12. Filipuzzi I, Steffen J, Germain M, Goepfert L, Conti MA, Potting C, Cerino R, Pfeifer M, Krastel P, Hoepfner D, Bastien J, Koehler CM, Helliwell SB. Stendomycin selectively inhibits TIM23-dependent mitochondrial protein import.. Nature chemical biology, 2017.
  13. Neal SE, Dabir DV, Wijaya J, Boon C, Koehler CM. Osm1 facilitates the transfer of electrons from Erv1 to fumarate in the redox-regulated import pathway in the mitochondrial intermembrane space.. Molecular biology of the cell, 2017.
  14. Sangwan S, Zhao A, Adams KL, Jayson CK, Sawaya MR, Guenther EL, Pan AC, Ngo J, Moore DM, Soriaga AB, Do TD, Goldschmidt L, Nelson R, Bowers MT, Koehler CM, Shaw DE, Novitch BG, Eisenberg DS. 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, 2017.
  15. Miyata N, Tang Z, Conti MA, Johnson ME, Douglas CJ, Hasson SA, Damoiseaux R, Chang CA, Koehler CM. Adaptation of a Genetic Screen Reveals an Inhibitor for Mitochondrial Protein Import Component Tim44.. The Journal of biological chemistry, 2017.
  16. Thangamani S, Maland M, Mohammad H, Pascuzzi PE, Avramova L, Koehler CM, Hazbun TR, Seleem MN. Repurposing Approach Identifies Auranofin with Broad Spectrum Antifungal Activity That Targets Mia40-Erv1 Pathway.. Frontiers in cellular and infection microbiology, 2017.
  17. Steffen J, Vashisht AA, Wan J, Jen JC, Claypool SM, Wohlschlegel JA, Koehler CM. Rapid degradation of mutant SLC25A46 by the ubiquitin-proteasome system results in MFN1/2-mediated hyperfusion of mitochondria.. Molecular biology of the cell, 2017.
  18. Wan J, Steffen J, Yourshaw M, Mamsa H, Andersen E, Rudnik-Schöneborn S, Pope K, Howell KB, McLean CA, Kornberg AJ, Joseph J, Lockhart PJ, Zerres K, Ryan MM, Nelson SF, Koehler CM, Jen JC. Loss of function of SLC25A46 causes lethal congenital pontocerebellar hypoplasia.. Brain : a journal of neurology, 2016.
  19. Wu TH, Sagullo E, Case D, Zheng X, Li Y, Hong JS, TeSlaa T, Patananan AN, McCaffery JM, Niazi K, Braas D, Koehler CM, Graeber TG, Chiou PY, Teitell MA. Mitochondrial Transfer by Photothermal Nanoblade Restores Metabolite Profile in Mammalian Cells.. Cell metabolism, 2016.
  20. Zhang J, Khvorostov I, Hong JS, Oktay Y, Vergnes L, Nuebel E, Wahjudi PN, Setoguchi K, Wang G, Do A, Jung HJ, McCaffery JM, Kurland IJ, Reue K, Lee WN, Koehler CM, Teitell MA. UCP2 regulates energy metabolism and differentiation potential of human pluripotent stem cells.. The EMBO journal, 2016.
  21. Lu YW, Galbraith L, Herndon JD, Lu YL, Pras-Raves M, Vervaart M, Van Kampen A, Luyf A, Koehler CM, McCaffery JM, Gottlieb E, Vaz FM, Claypool SM. Defining functional classes of Barth syndrome mutation in humans.. Human molecular genetics, 2016.
  22. Setoguchi K, TeSlaa T, Koehler CM, Teitell MA. P53 Regulates Rapid Apoptosis in Human Pluripotent Stem Cells.. Journal of molecular biology, 2015.
  23. Neal SE, Dabir DV, Tienson HL, Horn DM, Glaeser K, Ogozalek Loo RR, Barrientos A, Koehler CM. Mia40 Protein Serves as an Electron Sink in the Mia40-Erv1 Import Pathway.. The Journal of biological chemistry, 2015.
  24. Wang G, Shimada E, Nili M, Koehler CM, Teitell MA. Mitochondria-targeted RNA import.. Methods in molecular biology (Clifton, N.J.), 2015.
  25. Steffen J, Koehler CM. The great escape: Mgr2 of the mitochondrial TIM23 translocon is a gatekeeper Tasked with releasing membrane proteins.. Molecular cell, 2014.
  26. Xie Y, Zhang J, Lin Y, Gaeta X, Meng X, Wisidagama DR, Cinkornpumin J, Koehler CM, Malone CS, Teitell MA, Lowry WE. Defining the role of oxygen tension in human neural progenitor fate.. Stem cell reports, 2014.
  27. Miyata N, Steffen J, Johnson ME, Fargue S, Danpure CJ, Koehler CM. Pharmacologic rescue of an enzyme-trafficking defect in primary hyperoxaluria 1.. Proceedings of the National Academy of Sciences of the United States of America, 2014.
  28. 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. TMEM14C is required for erythroid mitochondrial heme metabolism.. The Journal of clinical investigation, 2014.
  29. 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, Merkenschlager A, Beblo S, Strom TM, Meitinger T, Freisinger P, Donati MA, Prokisch H, Mootha VK, DiMauro S, Paw BH. Macrocytic anemia and mitochondriopathy resulting from a defect in sideroflexin 4.. American journal of human genetics, 2013.
  30. Herndon JD, Claypool SM, Koehler CM. The Taz1p transacylase is imported and sorted into the outer mitochondrial membrane via a membrane anchor domain.. Eukaryotic cell, 2013.
  31. Koehler C. Carla Koehler: Small TIMs are a big deal. Interviewed by Caitlin Sedwick.. The Journal of cell biology, 2013.
  32. Dabir DV, Hasson SA, Setoguchi K, Johnson ME, Wongkongkathep P, Douglas CJ, Zimmerman J, Damoiseaux R, Teitell MA, Koehler CM. A small molecule inhibitor of redox-regulated protein translocation into mitochondria.. Developmental cell, 2013.
  33. Arango NA, Li L, Dabir D, Nicolau F, Pieretti-Vanmarcke R, Koehler C, McCarrey JR, Lu N, Donahoe PK. Meiosis I arrest abnormalities lead to severe oligozoospermia in meiosis 1 arresting protein (M1ap)-deficient mice.. Biology of reproduction, 2013.
  34. Levesque MP, Krauss J, Koehler C, Boden C, Harris MP. New tools for the identification of developmentally regulated enhancer regions in embryonic and adult zebrafish.. Zebrafish, 2013.
  35. Zhang J, Nuebel E, Daley GQ, Koehler CM, Teitell MA. Metabolic regulation in pluripotent stem cells during reprogramming and self-renewal.. Cell stem cell, 2012.
  36. von Ameln S, Wang G, Boulouiz R, Rutherford MA, Smith GM, Li Y, Pogoda HM, Nürnberg G, Stiller B, Volk AE, Borck G, Hong JS, Goodyear RJ, Abidi O, Nürnberg P, Hofmann K, Richardson GP, Hammerschmidt M, Moser T, Wollnik B, Koehler CM, Teitell MA, Barakat A, Kubisch C. A mutation in PNPT1, encoding mitochondrial-RNA-import protein PNPase, causes hereditary hearing loss.. American journal of human genetics, 2012.
  37. Bourens M, Dabir DV, Tienson HL, Sorokina I, Koehler CM, Barrientos A. Role of twin Cys-Xaa9-Cys motif cysteines in mitochondrial import of the cytochrome C oxidase biogenesis factor Cmc1.. The Journal of biological chemistry, 2012.
  38. Zhang J, Nuebel E, Wisidagama DR, Setoguchi K, Hong JS, Van Horn CM, Imam SS, Vergnes L, Malone CS, Koehler CM, Teitell MA. Measuring energy metabolism in cultured cells, including human pluripotent stem cells and differentiated cells.. Nature protocols, 2012.
  39. Wang G, Shimada E, Zhang J, Hong JS, Smith GM, Teitell MA, Koehler CM. Correcting human mitochondrial mutations with targeted RNA import.. Proceedings of the National Academy of Sciences of the United States of America, 2012.
  40. Zhang J, Khvorostov I, Hong JS, Oktay Y, Vergnes L, Nuebel E, Wahjudi PN, Setoguchi K, Wang G, Do A, Jung HJ, McCaffery JM, Kurland IJ, Reue K, Lee WN, Koehler CM, Teitell MA. UCP2 regulates energy metabolism and differentiation potential of human pluripotent stem cells.. The EMBO journal, 2011.
  41. Claypool SM, Koehler CM. The complexity of cardiolipin in health and disease.. Trends in biochemical sciences, 2011.
  42. Wang G, Shimada E, Koehler CM, Teitell MA. PNPASE and RNA trafficking into mitochondria.. Biochimica et biophysica acta, 2011.
  43. Claypool SM, Whited K, Srijumnong S, Han X, Koehler CM. Barth syndrome mutations that cause tafazzin complex lability.. The Journal of cell biology, 2011.
  44. Wang G, Chen HW, Oktay Y, Zhang J, Allen EL, Smith GM, Fan KC, Hong JS, French SW, McCaffery JM, Lightowlers RN, Morse HC, Koehler CM, Teitell MA. PNPASE regulates RNA import into mitochondria.. Cell, 2010.
  45. Curado S, Ober EA, Walsh S, Cortes-Hernandez P, Verkade H, Koehler CM, Stainier DY. The mitochondrial import gene tomm22 is specifically required for hepatocyte survival and provides a liver regeneration model.. Disease models & mechanisms, 2010.
  46. Hasson SA, Damoiseaux R, Glavin JD, Dabir DV, Walker SS, Koehler CM. Substrate specificity of the TIM22 mitochondrial import pathway revealed with small molecule inhibitor of protein translocation.. Proceedings of the National Academy of Sciences of the United States of America, 2010.
  47. Zaltsman Y, Shachnai L, Yivgi-Ohana N, Schwarz M, Maryanovich M, Houtkooper RH, Vaz FM, De Leonardis F, Fiermonte G, Palmieri F, Gillissen B, Daniel PT, Jimenez E, Walsh S, Koehler CM, Roy SS, Walter L, Hajnóczky G, Gross A. MTCH2/MIMP is a major facilitator of tBID recruitment to mitochondria.. Nature cell biology, 2010.
  48. Area-Gomez E, de Groof AJ, Boldogh I, Bird TD, Gibson GE, Koehler CM, Yu WH, Duff KE, Yaffe MP, Pon LA, Schon EA. Presenilins are enriched in endoplasmic reticulum membranes associated with mitochondria.. The American journal of pathology, 2009.
  49. Chacinska A, Koehler CM, Milenkovic D, Lithgow T, Pfanner N. Importing mitochondrial proteins: machineries and mechanisms.. Cell, 2009.
  50. Tienson HL, Dabir DV, Neal SE, Loo R, Hasson SA, Boontheung P, Kim SK, Loo JA, Koehler CM. Reconstitution of the mia40-erv1 oxidative folding pathway for the small tim proteins.. Molecular biology of the cell, 2009.
  51. Lu G, Sun H, Korge P, Koehler CM, Weiss JN, Wang Y. Functional characterization of a mitochondrial Ser/Thr protein phosphatase in cell death regulation.. Methods in enzymology, 2009.
  52. Claypool SM, Boontheung P, McCaffery JM, Loo JA, Koehler CM. The cardiolipin transacylase, tafazzin, associates with two distinct respiratory components providing insight into Barth syndrome.. Molecular biology of the cell, 2008.
  53. Claypool SM, Oktay Y, Boontheung P, Loo JA, Koehler CM. Cardiolipin defines the interactome of the major ADP/ATP carrier protein of the mitochondrial inner membrane.. The Journal of cell biology, 2008.
  54. Koehler CM, Tienson HL. Redox regulation of protein folding in the mitochondrial intermembrane space.. Biochimica et biophysica acta, 2008.
  55. Walsh S, Koehler CM. Gazing at translocation in the mitochondrion.. Cell, 2008.
  56. Beverly KN, Sawaya MR, Schmid E, Koehler CM. The Tim8-Tim13 complex has multiple substrate binding sites and binds cooperatively to Tim23.. Journal of molecular biology, 2008.
  57. Gebert N, Chacinska A, Wagner K, Guiard B, Koehler CM, Rehling P, Pfanner N, Wiedemann N. Assembly of the three small Tim proteins precedes docking to the mitochondrial carrier translocase.. EMBO reports, 2008.
  58. Chen HW, Koehler CM, Teitell MA. Human polynucleotide phosphorylase: location matters.. Trends in cell biology, 2007.
  59. Dabir DV, Leverich EP, Kim SK, Tsai FD, Hirasawa M, Knaff DB, Koehler CM. A role for cytochrome c and cytochrome c peroxidase in electron shuttling from Erv1.. The EMBO journal, 2007.
  60. Hwang DK, Claypool SM, Leuenberger D, Tienson HL, Koehler CM. Tim54p connects inner membrane assembly and proteolytic pathways in the mitochondrion.. The Journal of cell biology, 2007.
  61. Sedivá A, Smith CI, Asplund AC, Hadac J, Janda A, Zeman J, Hansíková H, Dvoráková L, Mrázová L, Velbri S, Koehler C, Roesch K, Sullivan KE, Futatani T, Ochs HD. Contiguous X-chromosome deletion syndrome encompassing the BTK, TIMM8A, TAF7L, and DRP2 genes.. Journal of clinical immunology, 2007.
  62. Lu G, Ren S, Korge P, Choi J, Dong Y, Weiss J, Koehler C, Chen JN, 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 & development, 2007.
  63. Stuart RA, Koehler CM. In vitro analysis of yeast mitochondrial protein import.. Current protocols in cell biology, 2007.
  64. Rainey RN, Glavin JD, Chen HW, French SW, Teitell MA, Koehler CM. A new function in translocation for the mitochondrial i-AAA protease Yme1: import of polynucleotide phosphorylase into the intermembrane space.. Molecular and cellular biology, 2006.
  65. Chen HW, Rainey RN, Balatoni CE, Dawson DW, Troke JJ, Wasiak S, Hong JS, McBride HM, Koehler CM, Teitell MA, French SW. Mammalian polynucleotide phosphorylase is an intermembrane space RNase that maintains mitochondrial homeostasis.. Molecular and cellular biology, 2006.
  66. French SW, Dawson DW, Chen HW, Rainey RN, Sievers SA, Balatoni CE, Wong L, Troke JJ, Nguyen MT, Koehler CM, Teitell MA. The TCL1 oncoprotein binds the RNase PH domains of the PNPase exoribonuclease without affecting its RNA degrading activity.. Cancer letters, 2006.
  67. Claypool SM, McCaffery JM, Koehler CM. Mitochondrial mislocalization and altered assembly of a cluster of Barth syndrome mutant tafazzins.. The Journal of cell biology, 2006.
  68. Koehler CM, Beverly KN, Leverich EP. Redox pathways of the mitochondrion.. Antioxidants & redox signaling, 2006.
  69. Claypool SM, Koehler CM. Hereditary spastic paraplegia: respiratory choke or unactivated substrate?. Cell, 2005.
  70. Likic VA, Perry A, Hulett J, Derby M, Traven A, Waller RF, Keeling PJ, Koehler CM, Curran SP, Gooley PR, Lithgow T. Patterns that define the four domains conserved in known and novel isoforms of the protein import receptor Tom20.. Journal of molecular biology, 2005.
  71. Curran SP, Leverich EP, Koehler CM, Larsen PL. Defective mitochondrial protein translocation precludes normal Caenorhabditis elegans development.. The Journal of biological chemistry, 2004.
  72. 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.
  73. Roesch K, Hynds PJ, Varga R, Tranebjaerg L, Koehler CM. The calcium-binding aspartate/glutamate carriers, citrin and aralar1, are new substrates for the DDP1/TIMM8a-TIMM13 complex.. Human molecular genetics, 2004.
  74. Koehler CM. New developments in mitochondrial assembly.. Annual review of cell and developmental biology, 2004.
  75. Koehler CM. The small Tim proteins and the twin Cx3C motif.. Trends in biochemical sciences, 2004.
  76. van der Bliek AM, Koehler CM. A mitochondrial rhomboid protease.. Developmental cell, 2003.
  77. Dyall SD, Lester DC, Schneider RE, Delgadillo-Correa MG, Plümper E, Martinez A, Koehler CM, Johnson PJ. Trichomonas vaginalis Hmp35, a putative pore-forming hydrogenosomal membrane protein, can form a complex in yeast mitochondria.. The Journal of biological chemistry, 2003.
  78. Leuenberger D, Curran SP, Wong D, Koehler CM. The role of Tim9p in the assembly of the TIM22 import complexes.. Traffic (Copenhagen, Denmark), 2003.
  79. 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.
  80. Roesch K, Curran SP, Tranebjaerg L, Koehler CM. Human deafness dystonia syndrome is caused by a defect in assembly of the DDP1/TIMM8a-TIMM13 complex.. Human molecular genetics, 2002.
  81. 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.
  82. Koehler CM. Protein translocation pathways of the mitochondrion.. FEBS letters, 2000.