School of Medicine

Pediatrics Bonkowsky Lab

Publications

1992-2016

Merrill ST, Nelson GR, Longo N, Bonkowsky JL. Cytotoxic edema and diffusion restriction as an early pathoradiologic marker in canavan disease: case report and review of the literature. Orphanet J Rare Dis. 2016 Dec 7;11(1):169.

Keefe MD, Bonkowsky JL. Transvection Arising from Transgene Interactions in Zebrafish. Zebrafish. 2016 Oct 25. [Epub ahead of print] PubMed PMID: 27779464.

Kang PB, Bale JF Jr, Mintz M, Joshi SM, Gilbert DL, Radabaugh C, Ruch-Ross H; Section on Neurology Executive Committee of the American Academy of Pediatrics, and the Board of Directors of the Child Neurology Society. The child neurology clinical workforce in 2015: Report of the AAP/CNS Joint Taskforce. Neurology. 2016 Aug 26. pii: 10.1212/WNL.0000000000003147. [Epub ahead of print]

Chen YC, Semenova S, Rozov S, Sundvik M, Bonkowsky JL, Panula P. A novel developmental role for dopaminergic signaling to specify hypothalamic neurotransmitter identity. J Biol Chem. 2016 Aug 18. pii: jbc.M115.697466. [Epub ahead of print]

Dukes AA, Bai Q, Van Laar VS, Zhou Y, Ilin V, David CN, Agim ZS, Bonkowsky JL, Cannon JR, Watkins SC, Croix CM, Burton EA, Berman SB. Live imaging of mitochondrial dynamics in CNS dopaminergic neurons in vivo demonstrates early reversal of mitochondrial transport following MPP(+) exposure. Neurobiology of Disease. 2016 Jul 21.

Tieder JS, Bonkowsky JL, Etzel RA, Franklin WH, Gremse DA, Herman B, Katz ES,  Krilov LR, Merritt JL 2nd, Norlin C, Percelay J, Sapién RE, Shiffman RN, Smith MB; SUBCOMMITTEE ON APPARENT LIFE THREATENING EVENTS. Brief Resolved Unexplained Events (Formerly Apparent Life-Threatening Events) and Evaluation of Lower-Risk Infants: Executive Summary. Pediatrics. 2016 May;137(5).

Brief Resolved Unexplained Events (Formerly Apparent Life-Threatening Events) and Evaluation of Lower-Risk Infants: Executive Summary.  Joel S. Tieder, Bonkowsky JL, Ruth A. Etzel, Wayne H. Franklin, David A. Gremse, Bruce Herman, Eliot S. Katz, Leonard R. Krilov, J. Lawrence Merritt, Chuck Norlin, Jack Percelay, Robert E. Sapién, Richard N. Shiffman, Michael B.H. Smith, SUBCOMMITTEE ON APPARENT LIFE THREATENING EVENTS.  Pediatrics Apr 2016, DOI: 10.1542/peds.2016-0591

Eisenhoffer GT, Slattum G, Ruiz OE, Otsuna H, Bryan CD, Lopez J, Wagner DS, Bonkowsky JL, Chien CB, Dorsky RI, Rosenblatt J. A toolbox to study epidermal cell types in zebrafish. J Cell Sci. 2016 May 5.

Milash B, Gao J, Stevenson TJ, Son JH, Dahl T, Bonkowsky JL. Temporal Dysynchrony in brain connectivity gene expression following hypoxia. BMC Genomics. 2016 May 4;17(1):334.

Helman G, Bonkowsky JL, Vanderver A. Neurologist Comfort in the Use of Next-Generation Sequencing Diagnostics: Current State and Future Prospects. JAMA Neurol. 2016 Apr 4.

Ostrander B, Bardsley T, Korgenski EK, Greene T, Bonkowsky JL. Neonatal Magnesium Levels Between 24 and 48 Hours of Life and Outcomes for Epilepsy and Motor Impairment in Premature Infants. Pediatr Neurol. 2016 Mar 3. pii: S0887-8994(15)30232-0.

Sivachenko A, Gordon HB, Kimball SS, Gavin EJ, Bonkowsky JL, Letsou A. Neurodegeneration in a Drosophila model of Adrenoleukodystrophy: the roles of the bubblegum and double bubble acyl-CoA synthetases. Disease Models and Mechanisms. 2016 Feb 18. pii: dmm.022244. [Epub ahead of print]

Bonkowsky JL. Shedding light on the leukodystrophies. Developmental Medicine and Child Neurology. 2016 Feb 15. doi: 10.1111/dmcn.13019. [Epub ahead of print]

McPherson AD, Barrios JP, Luks-Morgan SJ, Manfredi JP, Bonkowsky JL, Douglass  AD, Dorsky RI. Motor Behavior Mediated by Continuously Generated Dopaminergic Neurons in the Zebrafish Hypothalamus Recovers after Cell Ablation. Current Biology. 2016 Jan 25;26(2):263-9.

Son JH, Keefe MD, Stevenson TJ, Barrios JP, Anjewierden S, Newton JB, Douglass AD, Bonkowsky JL. Transgenic FingRs for Live Mapping of Synaptic Dynamics in Genetically-Defined Neurons. Scientific Reports (Nature). 2016 Jan 5;6:18734.

Nelson GR, Bonkowsky JL, Doll E, Green M, Hedlund GL, Moore KR, Bale JF Jr. Recognition and Management of Acute Flaccid Myelitis in Children. Pediatric Neurology. 2016 Feb;55:17-21.

Duncan RN, Xie Y, McPherson AD, Taibi AV, Bonkowsky JL, Douglass AD, Dorsky RI. Hypothalamic radial glia function as self-renewing neural progenitors in the  absence of Wnt/β-catenin signaling. Development. 2016 Jan 1;143(1):45-53.

Richards J, Korgenski EK, Srivastava R, Bonkowsky JL. Costs of the diagnostic odyssey in children with inherited leukodystrophies. Neurology. 2015 Sep 29;85(13):1167-70. Epub 2015 Aug 28.

Roundy LM, Filloux FM, Kerr L, Rimer A, Bonkowsky JL. Seizure Action Plans Do Not Reduce Health Care Utilization in Pediatric Epilepsy Patients. J Child Neurol. 2015 Aug 5.

Richards J, Korgenski EK, Taft RJ, Vanderver A, Bonkowsky JL. Targeted leukodystrophy diagnosis based on charges and yields for testing. Am J Med Genet A. 2015 Nov;167(11):2541-3.

Samuel R, Stephenson R, Roy P, Pryor R, Zhou L, Bonkowsky JL, Gale BK. Microfluidic-aided genotyping of zebrafish in the first 48 h with 100% viability. Biomed Microdevices. 2015 Apr;17(2):43.

Van Haren K, Bonkowsky JL, Bernard G, Murphy JL, Pizzino A, Helman G, Suhr D, Waggoner J, Hobson D, Vanderver A, Patterson MC; GLIA Consortium. Consensus statement on preventive and symptomatic care of leukodystrophy patients. Mol Genet Metab. 2015 Apr;114(4):516-26.

Doll E, Wilkes J, Cook LJ, Korgenski EK, Faix RG, Yoder BA, Srivastava R, Sherwin CM, Spigarelli MG, Clark EA, Bonkowsky JL. Neonatal magnesium levels correlate with motor outcomes in premature infants: a long-term retrospective cohort study. Front Pediatr. 2014 Nov 5;2:120.

Gordon HB, Letsou A, Bonkowsky JL. The leukodystrophies. Semin Neurol. 2014 Jul;34(3):312-20.

Anderson HM, Wilkes J, Korgenski EK, Pulsipher MA, Blaschke AJ, Hersh AL, Srivastava R, Bonkowsky JL. Preventable Infections in Children with Leukodystrophy. Ann Clin Transl Neurol. 2014 May 1;1(5):370-374.

Purnell S, Bleyl S, Bonkowsky JL. Clinical Exome Sequencing Identifies a Novel TUBB4A Mutation in a Child with Static Hypomyelinating Leukodystrophy. Pediatric Neurology. 2014. Jun;50(6):608-11.

Butterfield RJ, Stevenson TJ, Xing L, Newcomb TM, Nelson B, Zeng W, Li X, Lu HM, Lu H, Farwell Gonzalez KD, Wei JP, Chao EC, Prior TW, Snyder PJ, Bonkowsky JL, Swoboda KJ. Congenital lethal motor neuron disease with a novel defect in ribosome biogenesis. Neurology. 2014 Apr 15;82(15):1322-30.

Xing, L., Quist, T., Stevenson, T., Dahlem, T., Bonkowsky JL. Rapid and Efficient Zebrafish Genotyping Using PCR with High-resolution Melt Analysis. J. Vis. Exp. 2014. (), e51138, doi:10.3791/51138.

Brimley CJ, Lopez J, van Haren K, Wilkes J, Sheng X, Nelson C, Korgenski EK, Srivastava R, Bonkowsky JL. National Variation in Costs and Mortality for Leukodystrophy Patients in US Children's Hospitals. Pediatr Neurol. 2013 Sep;49(3):156-162.

Iwasaki K, Taguchi M, Bonkowsky JL, Kuwada JY. Expression of arginine vasotocin receptors in the developing zebrafish CNS. Gene Expr Patterns. 2013 Jul 2;13(8):335-342.

Tieder JS, Altman RL, Bonkowsky JL, Brand DA, Claudius I, Cunningham DJ, Dewolfe C, Percelay JM, Pitetti RD, Smith MB. Management of Apparent Life-Threatening Events in Infants: A Systematic Review. Journal of Pediatrics. 2013 Feb 14. doi:pii: S0022-3476(12)01575-2.

Schweitzer J, Löhr H, Bonkowsky JL, Hübscher K, Driever W. Sim1a and Arnt2 contribute to hypothalamo-spinal axon guidance by regulating Robo2 activity via a Robo3-dependent mechanism. Development. 2013 Jan;140(1):93-106.

Nelson C, Mundorff MB, Korgenski EK, Brimley CJ, Srivastava R, and Bonkowsky JL. Determinants of health care utilization in pediatric leukodystrophy patients. Journal of Pediatrics. Mar;162(3):624-628.e1.

Hoki R, Bonkowsky JL, Minich LL, Srivastava R, Pinto NM. Cardiac testing and outcomes in infants after an apparent life-threatening event. Arch Dis Child. 2012 Dec;97(12):1034-8.

Xing L, Hoshijima K, Grunwald DJ, Fujimoto E, Quist TS, Sneddon J, Chien CB, Stevenson TJ, Bonkowsky JL. Zebrafish foxP2 zinc finger nuclease mutant has normal axon pathfinding. PLoS One. 2012. 7(8):e43968.

Lambert AM, Bonkowsky JL, and Masino MA. The dopaminergic diencephalospinal tract mediates a developmental switch in the locomotor pattern of larval zebrafish. Journal of Neuroscience. 2012. 32: 13488-13500

Zimbric G, Bonkowsky JL, Jackson WD, Maloney CJ, Srivastava R. Adverse outcomes associated with gastroesophageal reflux disease are rare following an apparent life-threatening event. Journal of Hospital Medicine. 2012. 7(6):476-81.

Stevenson TJ, Trinh T, Kogelschatz C, Fujimoto E, Lush ME, Piotrowski T, Brimley CJ, Bonkowsky JL. Hypoxia disruption of vertebrate CNS pathfinding through EphrinB2 is rescued by magnesium. PLoS Genetics. 2012 Apr;8(4):e1002638. PMID: 22511881

Lakhina V, Maraccio C, Shao X, Lush M, Jain R, Fujimoto E, Bonkowsky J, Granato M, Raper J. Netrin/DCC signaling guides olfactory sensory axons to their correct location in the olfactory bulb. Journal of Neuroscience. 2012 Mar 28;32(13):4440-56. PMID: 22457493

Amir-Zilberstein L, Blechman J, Sztainberg Y, Norton WJH, Reuveny A, Borodovsky N, Tahor M, Bonkowsky JL, Bally-Cuif L, Chen A, Levkowitz G. Homeodomain protein Otp and activity-dependent splicing modulate neuronal adaptation to stress. Neuron. 2012. 26;73(2):279-91.

Berry JG, Poduri A, Bonkowsky JL, Zhou J, Graham DA, Welch C, Putney H, Srivastava R. The Impact of Children with Neurological Impairment on the U.S. Inpatient Healthcare System:· A Cohort Study. PLoS Medicine. 2012. 9(1):e1001158.

Gutnick, A, Blechman J, Kaslin J, Affolter M, Bonkowky JL, Levkowitz G. The hypothalamic neuropeptide oxytocin is required for formation of the neuro-vascular interface of the pituitary. Developmental Cell. 2011. 18:642-54.

Fujimoto E, Gaynes B, Brimley CJ, Chien CB, Bonkowsky JL. Gal80 Intersectional Regulation of Cell-Type Specific Expression in Vertebrates. Developmental Dynamics. 2011 Oct;240(10):2324-34.

Willis MW, Bonkowsky JL, Srivastava R, Grimmer JF. Usefulness of airway evaluation in infants initially seen with an apparent life-threatening event. Archives Otolaryngology Head & Neck Surgery. 2011. 137(4):359-62.

Fujimoto E, Stevenson TJ, Chien CB, Bonkowsky JL. Identification of a dopaminergic enhancer indicates complexity in vertebrate dopamine neuron phenotype specification. Developmental Biology. 2011. 352(2):393-404.

Guenther E, Powers A, Srivastava R, Bonkowsky JL. Abusive head trauma in children presenting with an apparent life-threatening event. Journal of Pediatrics. 2010. 157(5):821-5.

Ekstrand JJ, Herbener A, Rawlings J, Turney B, Ampofo K, Korgenski EK, Bonkowsky JL. Heightened neurologic complications in children with pandemic H1N1 influenza. Annals of Neurology. 2010. 68(5):762-6.

Bonkowsky JL, Nelson C, Kingston JL, Filloux FM, Mundorff MB, Srivastava R. The burden of inherited leukodystrophies in children. Neurology. 2010. 75(8):718-25.

Bonkowsky JL, Guenther E, Srivastava R, Filloux FM. Seizures in children following an apparent life-threatening event. Journal of Child Neurology. 2009. 24(6):709-13.

Kastenhuber, E., Kern, U., Bonkowsky, J.L., Chien, C.-B., Driever, W., and Schweitzer, J. Netrin-DCC, Robo-Slit and HSPGs coordinate lateral positioning of longitudinal dopaminergic diencephalospinal axons. Journal of Neuroscience. 2009. 29:8914-26.

Bonkowsky, J.L., Wang, X., Fujimoto, E., Lee, E.J., Chien, C.-B., and Dorsky, R.I. Domain-specific regulation of foxP2 CNS expression by lef1. BMC Developmental Biology. 2008. 8:103.

Bonkowsky, J.L., Guenther, E. Filloux, F.M, and Srivastava, R. Death, child abuse, and adverse neurological outcomes following an apparent life-threatening event. Pediatrics. 2008. 122:125-31.

Bonkowsky, J.L., and Chien, C.-B. Isolation and cloning of zebrafish foxP2. Developmental Dynamics. 2005. 234:740-6.

Bonkowsky, J.L., Frazer, J.K., Buchi, K.F., and Byington, C.L. Metamizole use by Latino immigrants: a common and potentially harmful home remedy. Pediatrics, 2002. 109: e98.

Duke, T., Blaschke, A.J., Sialis, S., and Bonkowsky, J.L. Hypoxaemia in acute respiratory and non-respiratory illnesses in neonates and children in a developing country. Archives of Disease in Childhood, 2002. 86: 108-12.

Yoshikawa*, S., Bonkowsky*, J.L., Kokel, M., Shyn, S., and Thomas, J.B. The Derailed guidance receptor does not require kinase activity in vivo. Journal of Neuroscience, 2001. 21: RC119.

Bonkowsky*, J.L, Yoshikawa*, S., O’Keefe, D.D., Scully, A.L., and Thomas, J.B. Axon routing across the midline controlled by the Drosophila Derailed receptor. Nature, 1999. 402: 540-4.

Bonkowsky, J.L., and Thomas, J.B. Cell-type specific modular regulation of derailed in the Drosophila nervous system. Mechanisms of Development, 1998. 82: 181-4.

Oates, A.C., Bonkowsky, J.L., Irvine, D.V., Kelly, L.E., Thomas, J.B., and Wilks, A.F. Embryonic expression and activity of doughnut, a second RYK homolog in Drosophila. Mechanisms of Development, 1998. 78: 165-9.

Callahan, C.A., Bonkovsky, J.L., Scully, A.L., and Thomas, J.B. Derailed is required for muscle attachment site selection in Drosophila. Development, 1996. 122: 2761-67.

Moll, T., Dirick, L., Auer, H., Bonkovsky, J., and Nasmyth, K. SWI6 is a regulatory subunit of two different cell-cycle START-dependent transcription factors in S. cerevisiae. Journal of Cell Science, 1992. 16: 87-96.