Research ArticlePYRUVATE DEHYDROGENASE DEFICIENCY

Brain metabolism modulates neuronal excitability in a mouse model of pyruvate dehydrogenase deficiency

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Science Translational Medicine  20 Feb 2019:
Vol. 11, Issue 480, eaan0457
DOI: 10.1126/scitranslmed.aan0457

Article Information

vol. 11 no. 480

PubMed: 
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History: 
  • Received for publication March 2, 2017
  • Resubmitted September 25, 2018
  • Accepted for publication January 31, 2019
  • .

Author Information

  1. Vikram Jakkamsetti1,2,*,
  2. Isaac Marin-Valencia1,2,3,*,
  3. Qian Ma1,2,*,
  4. Levi B. Good1,2,,
  5. Tyler Terrill1,2,
  6. Karthik Rajasekaran1,2,
  7. Kumar Pichumani4,,
  8. Chalermchai Khemtong4,5,
  9. M. Ali Hooshyar6,
  10. Chandrasekhar Sundarrajan1,2,
  11. Mulchand S. Patel7,
  12. Robert M. Bachoo2,8,
  13. Craig R. Malloy4,5,8 and
  14. Juan M. Pascual1,2,9,10,11,§
  1. 1Rare Brain Disorders Program, University of Texas Southwestern Medical Center, Dallas, TX 75390, USA.
  2. 2Department of Neurology and Neurotherapeutics, University of Texas Southwestern Medical Center, Dallas, TX 75390, USA.
  3. 3Laboratory of Developmental Neurobiology, The Rockefeller University, New York, NY 10065, USA.
  4. 4Advanced Imaging Research Center, University of Texas Southwestern Medical Center, Dallas, TX 75390, USA.
  5. 5Department of Radiology, University of Texas Southwestern Medical Center, Dallas, TX 75390, USA.
  6. 6Department of Mathematical Sciences, The University of Texas at Dallas, Richardson, TX 75080, USA.
  7. 7Department of Biochemistry, SUNY Buffalo, Buffalo, NY 14203, USA.
  8. 8Department of Internal Medicine, University of Texas Southwestern Medical Center, Dallas, TX 75390, USA.
  9. 9Department of Physiology, University of Texas Southwestern Medical Center, Dallas, TX 75390, USA.
  10. 10Department of Pediatrics, University of Texas Southwestern Medical Center, Dallas, TX 75390, USA.
  11. 11Eugene McDermott Center for Human Growth & Development / Center for Human Genetics, University of Texas Southwestern Medical Center, Dallas, TX 75390, USA.
  1. §Corresponding author. Email: juan.pascual{at}utsouthwestern.edu
  • * These authors contributed equally to this work.

  • Present address: Department of Biomedical Engineering, Louisiana Tech University, Ruston, LA 71272, USA.

  • Present address: Department of Neurosurgery, Houston Methodist Hospital and Research Institute, Houston, Houston, TX 77030, USA.

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