Northwestern University Feinberg School of Medicine
Ozdinler Lab
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Publications

Learn more about the work that we do in the Pembe Hande Ozdinler Laboratory through our recent publications.
  1. Elmansy MF, Reidl CT, Rahaman M, Özdinler PH, Silverman RB  Small molecules targeting different cellular pathologies for the treatment of amyotrophic lateral sclerosis.  Med Res Rev  2023 Nov;43(6):2260-2302. doi:10.1002/med.21974
  2. Helmold BR, Pauss KE, Ozdinler PH  TDP-43 protein interactome informs about perturbed canonical pathways and may help develop personalized medicine approaches for patients with TDP-43 pathology.  Drug Discov Today  2023 Sep 14;28(11):103769. doi:10.1016/j.drudis.2023.103769
  3. Gautam M, Genç B, Helmold B, Ahrens A, Kuka J, Makrecka-Kuka M, Günay A, Koçak N, Aguilar-Wickings IR, Keefe D  et al.  SBT-272 improves TDP-43 pathology in ALS upper motor neurons by modulating mitochondrial integrity, motility, and function.  Neurobiol Dis  2023 Mar;178:106022. doi:10.1016/j.nbd.2023.106022
  4. Kirsanov O, Johnson T, Malachowski T, Niedenberger BA, Gilbert EA, Bhowmick D, Ozdinler PH, Gray DA, Fisher-Wellman K, Hermann BP  et al.  Modeling mammalian spermatogonial differentiation and meiotic initiation in vitro.  Development  2022 Nov 15;149(22). pii:dev200713
  5. Genç B, Jara JH, Sanchez SS, Lagrimas AKB, Gözütok Ö, Koçak N, Zhu Y, Hande Özdinler P  Upper motor neurons are a target for gene therapy and UCHL1 is necessary and sufficient to improve cellular integrity of diseased upper motor neurons.  Gene Ther  2022 Apr;29(3-4):178-192. doi:10.1038/s41434-021-00303-4
  6. Genç B, Gautam M, Helmold BR, Koçak N, Günay A, Goshu GM, Silverman RB, Hande Ozdinler P  NU-9 improves health of hSOD1G93A mouse upper motor neurons in vitro, especially in combination with riluzole or edaravone.  Sci Rep  2022 Mar 30;12(1):5383. pii:5383
  7. Gautam M, Gunay A, Chandel NS, Ozdinler PH  Mitochondrial dysregulation occurs early in ALS motor cortex with TDP-43 pathology and suggests maintaining NAD+ balance as a therapeutic strategy.  Sci Rep  2022 Mar 11;12(1):4287. pii:4287
  8. Gautam M, Carratore RD, Helmold B, Tessa A, Gozutok O, Chandel N, Idrisoglu H, Bongioanni P, Battini R, Ozdinler PH  2-Year-Old and 3-Year-Old Italian ALS Patients with Novel ALS2 Mutations: Identification of Key Metabolites in Their Serum and Plasma.  Metabolites  2022 Feb 12;12(2). pii:174
  9. Gozutok O, Helmold BR, Ozdinler PH  Mutations and Protein Interaction Landscape Reveal Key Cellular Events Perturbed in Upper Motor Neurons with HSP and PLS.  Brain Sci  2021 Apr 29;11(5). pii:578
  10. Gunay A, Shin HH, Gozutok O, Gautam M, Ozdinler PH  Importance of lipids for upper motor neuron health and disease.  Semin Cell Dev Biol  2021 Apr;112:92-104. doi:10.1016/j.semcdb.2020.11.004
  11. Özdinler PH  Expanded access: opening doors to personalized medicine for rare disease patients and patients with neurodegenerative diseases.  FEBS J  2021 Mar;288(5):1457-1461. doi:10.1111/febs.15529
  12. Genç B, Gautam M, Gözütok Ö, Dervishi I, Sanchez S, Goshu GM, Koçak N, Xie E, Silverman RB, Özdinler PH  Improving mitochondria and ER stability helps eliminate upper motor neuron degeneration that occurs due to mSOD1 toxicity and TDP-43 pathology.  Clin Transl Med  2021 Feb;11(2):e336. pii:e336
  13. Ozdinler PH, Gautam M, Gozutok O, Konrad C, Manfredi G, Area Gomez E, Mitsumoto H, Erb ML, Tian Z, Haase G  Better understanding the neurobiology of primary lateral sclerosis.  Amyotroph Lateral Scler Frontotemporal Degener  2020 Nov;21(sup1):35-46. doi:10.1080/21678421.2020.1837175
  14. Özdinler PH  Help from peripheral macrophages in ALS?  Nat Neurosci  2020 Nov;23(11):1311-1312. doi:10.1038/s41593-020-00727-y
  15. Jara JH, Sheets PL, Nigro MJ, Perić M, Brooks C, Heller DB, Martina M, Andjus PR, Ozdinler PH  The Electrophysiological Determinants of Corticospinal Motor Neuron Vulnerability in ALS.  Front Mol Neurosci  2020;13:73. pii:73
  16. Genc B, Gozutok O, Kocak N, Ozdinler PH  The Timing and Extent of Motor Neuron Vulnerability in ALS Correlates with Accumulation of Misfolded SOD1 Protein in the Cortex and in the Spinal Cord.  Cells  2020 Feb 22;9(2). pii:502
  17. Gautam M, Xie EF, Kocak N, Ozdinler PH  Mitoautophagy: A Unique Self-Destructive Path Mitochondria of Upper Motor Neurons With TDP-43 Pathology Take, Very Early in ALS.  Front Cell Neurosci  2019;13:489. pii:489
  18. Jara JH, Gautam M, Kocak N, Xie EF, Mao Q, Bigio EH, Özdinler PH  MCP1-CCR2 and neuroinflammation in the ALS motor cortex with TDP-43 pathology.  J Neuroinflammation  2019 Oct 30;16(1):196. pii:196
  19. Genc B, Gozutok O, Ozdinler PH  Complexity of Generating Mouse Models to Study the Upper Motor Neurons: Let Us Shift Focus from Mice to Neurons.  Int J Mol Sci  2019 Aug 07;20(16). pii:3848
  20. Gautam M, Jara JH, Kocak N, Rylaarsdam LE, Kim KD, Bigio EH, Hande Özdinler P  Mitochondria, ER, and nuclear membrane defects reveal early mechanisms for upper motor neuron vulnerability with respect to TDP-43 pathology.  Acta Neuropathol  2019 Jan;137(1):47-69. doi:10.1007/s00401-018-1934-8
  21. Dervishi I, Gozutok O, Murnan K, Gautam M, Heller D, Bigio E, Ozdinler PH  Protein-protein interactions reveal key canonical pathways, upstream regulators, interactome domains, and novel targets in ALS.  Sci Rep  2018 Oct 03;8(1):14732. pii:14732
  22. Dervishi I, Ozdinler PH  Incorporating upper motor neuron health in ALS drug discovery.  Drug Discov Today  2018 Mar;23(3):696-703. doi:10.1016/j.drudis.2018.01.027
  23. Jara JH, Genç B, Stanford MJ, Pytel P, Roos RP, Weintraub S, Mesulam MM, Bigio EH, Miller RJ, Özdinler PH  Evidence for an early innate immune response in the motor cortex of ALS.  J Neuroinflammation  2017 Jun 26;14(1):129. pii:129
  24. Fil D, DeLoach A, Yadav S, Alkam D, MacNicol M, Singh A, Compadre CM, Goellner JJ, O'Brien CA, Fahmi T  et al.  Mutant Profilin1 transgenic mice recapitulate cardinal features of motor neuron disease.  Hum Mol Genet  2017 Feb 15;26(4):686-701. pii:ddw429
  25. Genç B, Jara JH, Lagrimas AK, Pytel P, Roos RP, Mesulam MM, Geula C, Bigio EH, Özdinler PH  Apical dendrite degeneration, a novel cellular pathology for Betz cells in ALS.  Sci Rep  2017 Feb 06;7:41765. pii:41765
  26. Brent JR, Ozdinler PH  Deciphering the molecular logic of ALS using model organisms: "A family affair.".  SOJ Neurol  2017;4(1):1-3. doi:10.15226/2374-6858/4/1/00132
  27. Geevasinga N, Menon P, Özdinler PH, Kiernan MC, Vucic S  Pathophysiological and diagnostic implications of cortical dysfunction in ALS.  Nat Rev Neurol  2016 Nov;12(11):651-661. doi:10.1038/nrneurol.2016.140
  28. Labuzzetta CJ, Antonio ML, Watson PM, Wilson RC, Laboissonniere LA, Trimarchi JM, Genc B, Ozdinler PH, Watson DK, Anderson PE  Complementary feature selection from alternative splicing events and gene expression for phenotype prediction.  Bioinformatics  2016 Sep 01;32(17):i421-i429. doi:10.1093/bioinformatics/btw430
  29. Genç B, Jara JH, Schultz MC, Manuel M, Stanford MJ, Gautam M, Klessner JL, Sekerkova G, Heller DB, Cox GA  et al.  Absence of UCHL 1 function leads to selective motor neuropathy.  Ann Clin Transl Neurol  2016 May;3(5):331-45. doi:10.1002/acn3.298
  30. Gautam M, Jara JH, Sekerkova G, Yasvoina MV, Martina M, Özdinler PH  Absence of alsin function leads to corticospinal motor neuron vulnerability via novel disease mechanisms.  Hum Mol Genet  2016 Mar 15;25(6):1074-87. doi:10.1093/hmg/ddv631
  31. Jara JH, Stanford MJ, Zhu Y, Tu M, Hauswirth WW, Bohn MC, DeVries SH, Özdinler PH  Healthy and diseased corticospinal motor neurons are selectively transduced upon direct AAV2-2 injection into the motor cortex.  Gene Ther  2016 Mar;23(3):272-82. doi:10.1038/gt.2015.112
  32. Klionsky DJ, Abdelmohsen K, Abe A, Abedin MJ, Abeliovich H, Acevedo Arozena A, Adachi H, Adams CM, Adams PD, Adeli K  et al.  Guidelines for the use and interpretation of assays for monitoring autophagy (3rd edition).  Autophagy  2016;12(1):1-222. doi:10.1080/15548627.2015.1100356
  33. Jara JH, Genç B, Cox GA, Bohn MC, Roos RP, Macklis JD, Ulupınar E, Özdinler PH  Corticospinal Motor Neurons Are Susceptible to Increased ER Stress and Display Profound Degeneration in the Absence of UCHL1 Function.  Cereb Cortex  2015 Nov;25(11):4259-72. doi:10.1093/cercor/bhu318
  34. Genç B, Lagrimas AK, Kuru P, Hess R, Tu MW, Menichella DM, Miller RJ, Paller AS, Özdinler PH  Visualization of Sensory Neurons and Their Projections in an Upper Motor Neuron Reporter Line.  PLoS One  2015;10(7):e0132815. pii:e0132815
  35. Joyce PI, Mcgoldrick P, Saccon RA, Weber W, Fratta P, West SJ, Zhu N, Carter S, Phatak V, Stewart M  et al.  A novel SOD1-ALS mutation separates central and peripheral effects of mutant SOD1 toxicity.  Hum Mol Genet  2015 Apr 01;24(7):1883-97. doi:10.1093/hmg/ddu605
  36. Ozdinler PH, Silverman RB  Treatment of amyotrophic lateral sclerosis: lessons learned from many failures.  ACS Med Chem Lett  2014 Nov 13;5(11):1179-81. doi:10.1021/ml500404b
  37. Genç B, Özdinler PH  Moving forward in clinical trials for ALS: motor neurons lead the way please.  Drug Discov Today  2014 Apr;19(4):441-9. doi:10.1016/j.drudis.2013.10.014
  38. Jara JH, Genç B, Klessner JL, Ozdinler PH  Retrograde labeling, transduction, and genetic targeting allow cellular analysis of corticospinal motor neurons: implications in health and disease.  Front Neuroanat  2014;8:16. pii:16
  39. Jara JH, Frank DD, Özdinler PH  Could dysregulation of UPS be a common underlying mechanism for cancer and neurodegeneration? Lessons from UCHL1.  Cell Biochem Biophys  2013 Sep;67(1):45-53. doi:10.1007/s12013-013-9631-7
  40. Yasvoina MV, Genç B, Jara JH, Sheets PL, Quinlan KA, Milosevic A, Shepherd GM, Heckman CJ, Özdinler PH  eGFP expression under UCHL1 promoter genetically labels corticospinal motor neurons and a subpopulation of degeneration-resistant spinal motor neurons in an ALS mouse model.  J Neurosci  2013 May 01;33(18):7890-904. doi:10.1523/JNEUROSCI.2787-12.2013
  41. Jara JH, Villa SR, Khan NA, Bohn MC, Ozdinler PH  AAV2 mediated retrograde transduction of corticospinal motor neurons reveals initial and selective apical dendrite degeneration in ALS.  Neurobiol Dis  2012 Aug;47(2):174-83. doi:10.1016/j.nbd.2012.03.036
  42. Srivastava DP, Woolfrey KM, Jones KA, Anderson CT, Smith KR, Russell TA, Lee H, Yasvoina MV, Wokosin DL, Ozdinler PH  et al.  An autism-associated variant of Epac2 reveals a role for Ras/Epac2 signaling in controlling basal dendrite maintenance in mice.  PLoS Biol  2012;10(6):e1001350. pii:e1001350
  43. Ozdinler PH, Benn S, Yamamoto TH, Güzel M, Brown RH, Macklis JD  Corticospinal motor neurons and related subcerebral projection neurons undergo early and specific neurodegeneration in hSOD1G⁹³A transgenic ALS mice.  J Neurosci  2011 Mar 16;31(11):4166-77. doi:10.1523/JNEUROSCI.4184-10.2011