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Kevin Ess
Assistant Professor of Neurology
Last active: 2/16/2016

Profile

Our research focuses on the neurobiology of progenitor cells, specifically the mechanisms by which neural progenitors make cell fate decisions during development. To study this we used mouse and zebrafish animal models as well as human induced pluripotent stem cells (iPSC) derived from patients with neurolgical diseases.

We have used the human genetic disease tuberous sclerosis complex (TSC) as a model system to investigate neural progenitor cell differentiation. TSC is due to inactivation of the TSC1 or TSC2 genes and is seen in approximately 1:6,000 people.

Multiple organs are affected in TSC though brain abnormalities generally cause the greatest suffering with many patients having seizure disorders (epilepsy), autism, developmental delay, psychiatric, and behavioral problems. These neurologic features are thought to be due to brain malformations called tubers.

Tubers have severe disruption of cortical layers and contain many abnormal large cells that express neuronal as well as glial markers. While multiple alterations have been discovered, dysregulation of the mTOR signaling appears to be a central abnormality. While these and other findings represent very exciting advances, much remains about the role of the TSC genes during normal development and how their mutation leads to the clinical manifestations of TSC.

Publications

The following timeline graph is generated from all co-authored publications.

Featured publications are shown below:

  1. Hypomyelination following deletion of Tsc2 in oligodendrocyte precursors. Carson RP, Kelm ND, West KL, Does MD, Fu C, Weaver G, McBrier E, Parker B, Grier MD, Ess KC (2015) Ann Clin Transl Neurol 2(12): 1041-54
    › Primary publication · 26734657 (PubMed) · PMC4693589 (PubMed Central)
  2. Dynamic tubers in tuberous sclerosis complex: A window for intervention? Ess KC, Chugani HT (2015) Neurology 85(18): 1530-1
    › Primary publication · 26432847 (PubMed)
  3. Evaluation of diffusion kurtosis imaging in ex vivo hypomyelinated mouse brains. Kelm ND, West KL, Carson RP, Gochberg DF, Ess KC, Does MD (2016) Neuroimage 124(Pt A): 612-626
    › Primary publication · 26400013 (PubMed) · PMC4651761 (PubMed Central)
  4. Combinatorial polymer matrices enhance in vitro maturation of human induced pluripotent stem cell-derived cardiomyocytes. Chun YW, Balikov DA, Feaster TK, Williams CH, Sheng CC, Lee JB, Boire TC, Neely MD, Bellan LM, Ess KC, Bowman AB, Sung HJ, Hong CC (2015) Biomaterials : 52-64
    › Primary publication · 26204225 (PubMed) · PMC4550551 (PubMed Central)
  5. A post-developmental genetic screen for zebrafish models of inherited liver disease. Kim SH, Wu SY, Baek JI, Choi SY, Su Y, Flynn CR, Gamse JT, Ess KC, Hardiman G, Lipschutz JH, Abumrad NN, Rockey DC (2015) PLoS One 10(5): e0125980
    › Primary publication · 25950913 (PubMed) · PMC4423964 (PubMed Central)
  6. Interaction with WDR5 promotes target gene recognition and tumorigenesis by MYC. Thomas LR, Wang Q, Grieb BC, Phan J, Foshage AM, Sun Q, Olejniczak ET, Clark T, Dey S, Lorey S, Alicie B, Howard GC, Cawthon B, Ess KC, Eischen CM, Zhao Z, Fesik SW, Tansey WP (2015) Mol Cell 58(3): 440-52
    › Primary publication · 25818646 (PubMed) · PMC4427524 (PubMed Central)
  7. Tuberous sclerosis associated neuropsychiatric disorders (TAND) and the TAND Checklist. de Vries PJ, Whittemore VH, Leclezio L, Byars AW, Dunn D, Ess KC, Hook D, King BH, Sahin M, Jansen A (2015) Pediatr Neurol 52(1): 25-35
    › Primary publication · 25532776 (PubMed) · PMC4427347 (PubMed Central)
  8. A novel manganese-dependent ATM-p53 signaling pathway is selectively impaired in patient-based neuroprogenitor and murine striatal models of Huntington's disease. Tidball AM, Bryan MR, Uhouse MA, Kumar KK, Aboud AA, Feist JE, Ess KC, Neely MD, Aschner M, Bowman AB (2015) Hum Mol Genet 24(7): 1929-44
    › Primary publication · 25489053 (PubMed) · PMC4355025 (PubMed Central)
  9. PARK2 patient neuroprogenitors show increased mitochondrial sensitivity to copper. Aboud AA, Tidball AM, Kumar KK, Neely MD, Han B, Ess KC, Hong CC, Erikson KM, Hedera P, Bowman AB (2015) Neurobiol Dis : 204-12
    › Primary publication · 25315681 (PubMed) · PMC4394022 (PubMed Central)
  10. Identification of a common Wnt-associated genetic signature across multiple cell types in pulmonary arterial hypertension. West JD, Austin ED, Gaskill C, Marriott S, Baskir R, Bilousova G, Jean JC, Hemnes AR, Menon S, Bloodworth NC, Fessel JP, Kropski JA, Irwin D, Ware LB, Wheeler L, Hong CC, Meyrick B, Loyd JE, Bowman AB, Ess KC, Klemm DJ, Young PP, Merryman WD, Kotton D, Majka SM (2014) Am J Physiol Cell Physiol 307(5): C415-30
    › Primary publication · 24871858 (PubMed) · PMC4154073 (PubMed Central)
  11. Gammaretroviral vector encoding a fluorescent marker to facilitate detection of reprogrammed human fibroblasts during iPSC generation. Srinivasakumar N, Zaboikin M, Tidball AM, Aboud AA, Neely MD, Ess KC, Bowman AB, Schuening FG (2013) PeerJ : e224
    › Primary publication · 24392288 (PubMed) · PMC3869187 (PubMed Central)
  12. Multi-organ abnormalities and mTORC1 activation in zebrafish model of multiple acyl-CoA dehydrogenase deficiency. Kim SH, Scott SA, Bennett MJ, Carson RP, Fessel J, Brown HA, Ess KC (2013) PLoS Genet 9(6): e1003563
    › Primary publication · 23785301 (PubMed) · PMC3681725 (PubMed Central)
  13. Patient heal thyself: modeling and treating neurological disorders using patient-derived stem cells. Ess KC (2013) Exp Biol Med (Maywood) 238(3): 308-14
    › Primary publication · 23598977 (PubMed) · PMC3784026 (PubMed Central)
  14. Heterozygous inactivation of tsc2 enhances tumorigenesis in p53 mutant zebrafish. Kim SH, Kowalski ML, Carson RP, Bridges LR, Ess KC (2013) Dis Model Mech 6(4): 925-33
    › Primary publication · 23580196 (PubMed) · PMC3701212 (PubMed Central)
  15. Autophagy induction is a Tor- and Tp53-independent cell survival response in a zebrafish model of disrupted ribosome biogenesis. Boglev Y, Badrock AP, Trotter AJ, Du Q, Richardson EJ, Parslow AC, Markmiller SJ, Hall NE, de Jong-Curtain TA, Ng AY, Verkade H, Ober EA, Field HA, Shin D, Shin CH, Hannan KM, Hannan RD, Pearson RB, Kim SH, Ess KC, Lieschke GJ, Stainier DY, Heath JK (2013) PLoS Genet 9(2): e1003279
    › Primary publication · 23408911 (PubMed) · PMC3567153 (PubMed Central)
  16. Conditional and domain-specific inactivation of the Tsc2 gene in neural progenitor cells. Fu C, Ess KC (2013) Genesis 51(4): 284-92
    › Primary publication · 23359422 (PubMed) · PMC3633697 (PubMed Central)
  17. Genetic risk for Parkinson's disease correlates with alterations in neuronal manganese sensitivity between two human subjects. Aboud AA, Tidball AM, Kumar KK, Neely MD, Ess KC, Erikson KM, Bowman AB (2012) Neurotoxicology 33(6): 1443-9
    › Primary publication · 23099318 (PubMed) · PMC3518601 (PubMed Central)
  18. Deletion of Rictor in neural progenitor cells reveals contributions of mTORC2 signaling to tuberous sclerosis complex. Carson RP, Fu C, Winzenburger P, Ess KC (2013) Hum Mol Genet 22(1): 140-52
    › Primary publication · 23049074 (PubMed) · PMC3522403 (PubMed Central)
  19. DMH1, a highly selective small molecule BMP inhibitor promotes neurogenesis of hiPSCs: comparison of PAX6 and SOX1 expression during neural induction. Neely MD, Litt MJ, Tidball AM, Li GG, Aboud AA, Hopkins CR, Chamberlin R, Hong CC, Ess KC, Bowman AB (2012) ACS Chem Neurosci 3(6): 482-91
    › Primary publication · 22860217 (PubMed) · PMC3400384 (PubMed Central)
  20. Cystogenesis and elongated primary cilia in Tsc1-deficient distal convoluted tubules. Armour EA, Carson RP, Ess KC (2012) Am J Physiol Renal Physiol 303(4): F584-92
    › Primary publication · 22674026 (PubMed) · PMC3423116 (PubMed Central)
  21. New therapies for tuber-less sclerosis: white matter matters? Ess KC, Roach ES (2012) Neurology 78(8): 520-1
    › Primary publication · 22262747 (PubMed)
  22. GABAergic interneuron development and function is modulated by the Tsc1 gene. Fu C, Cawthon B, Clinkscales W, Bruce A, Winzenburger P, Ess KC (2012) Cereb Cortex 22(9): 2111-9
    › Primary publication · 22021912 (PubMed) · PMC3412444 (PubMed Central)
  23. Neuronal and glia abnormalities in Tsc1-deficient forebrain and partial rescue by rapamycin. Carson RP, Van Nielen DL, Winzenburger PA, Ess KC (2012) Neurobiol Dis 45(1): 369-80
    › Primary publication · 21907282 (PubMed) · PMC3225598 (PubMed Central)
  24. Tuberous sclerosis complex: everything old is new again. Ess KC (2009) J Neurodev Disord 1(2): 141-9
    › Primary publication · 21547713 (PubMed) · PMC3164024 (PubMed Central)
  25. Zebrafish model of tuberous sclerosis complex reveals cell-autonomous and non-cell-autonomous functions of mutant tuberin. Kim SH, Speirs CK, Solnica-Krezel L, Ess KC (2011) Dis Model Mech 4(2): 255-67
    › Primary publication · 20959633 (PubMed) · PMC3046101 (PubMed Central)
  26. Tuberous sclerosis complex: a brave new world? Ess KC (2010) Curr Opin Neurol 23(2): 189-93
    › Primary publication · 20087180 (PubMed) · PMC2884012 (PubMed Central)
  27. Treatment of infantile spasms in tuberous sclerosis complex: dismal outcomes but future hope? Ess KC (2009) Nat Clin Pract Neurol 5(2): 72-3
    › Primary publication · 19107107 (PubMed)
  28. Abnormal glutamate homeostasis and impaired synaptic plasticity and learning in a mouse model of tuberous sclerosis complex. Zeng LH, Ouyang Y, Gazit V, Cirrito JR, Jansen LA, Ess KC, Yamada KA, Wozniak DF, Holtzman DM, Gutmann DH, Wong M (2007) Neurobiol Dis 28(2): 184-96
    › Primary publication · 17714952 (PubMed) · PMC2117357 (PubMed Central)
  29. The neurobiology of tuberous sclerosis complex. Ess KC (2006) Semin Pediatr Neurol 13(1): 37-42
    › Primary publication · 16818174 (PubMed)
  30. Developmental origin of subependymal giant cell astrocytoma in tuberous sclerosis complex. Ess KC, Kamp CA, Tu BP, Gutmann DH (2005) Neurology 64(8): 1446-9
    › Primary publication · 15851742 (PubMed)
  31. Expression profiling in tuberous sclerosis complex (TSC) knockout mouse astrocytes to characterize human TSC brain pathology. Ess KC, Uhlmann EJ, Li W, Li H, Declue JE, Crino PB, Gutmann DH (2004) Glia 46(1): 28-40
    › Primary publication · 14999811 (PubMed)
  32. Impaired glial glutamate transport in a mouse tuberous sclerosis epilepsy model. Wong M, Ess KC, Uhlmann EJ, Jansen LA, Li W, Crino PB, Mennerick S, Yamada KA, Gutmann DH (2003) Ann Neurol 54(2): 251-6
    › Primary publication · 12891680 (PubMed)
  33. Cerebrospinal fluid neuron-specific enolase following seizures in children: role of etiology. Wong M, Ess K, Landt M (2002) J Child Neurol 17(4): 261-4
    › Primary publication · 12088080 (PubMed)
  34. Diverse developing mouse lineages exhibit high-level c-Myb expression in immature cells and loss of expression upon differentiation. Ess KC, Witte DP, Bascomb CP, Aronow BJ (1999) Oncogene 18(4): 1103-11
    › Primary publication · 10023687 (PubMed)
  35. Winner of the Theodore E. Woodward Award: c-Myb and the coordinate regulation of thymic genes. Hutton JJ, Ess KC, Witte DP, Aronow BJ (1996) Trans Am Clin Climatol Assoc : 115-24
    › Primary publication · 8725565 (PubMed) · PMC2376566 (PubMed Central)
  36. Double-stranded phosphorothioate oligonucleotide modulation of gene expression. Ess KC, Hutton JJ, Aronow BJ (1994) Ann N Y Acad Sci : 321-3
    › Primary publication · 8024203 (PubMed)
  37. Suppression of tumorigenicity, but not invasion, in glioblastoma/HeLa cell hybrids. Ess K, Chen H, Kier A, Brackenbury R (1995) J Cell Physiol 162(3): 341-7
    › Primary publication · 7860642 (PubMed)
  38. A central role for a single c-Myb binding site in a thymic locus control region. Ess KC, Whitaker TL, Cost GJ, Witte DP, Hutton JJ, Aronow BJ (1995) Mol Cell Biol 15(10): 5707-15
    › Primary publication · 7565722 (PubMed) · PMC230821 (PubMed Central)