The Urea Cycle Disorders Consortium
Participating Clinical Centers  

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Baylor College of Medicine
Houston, Texas

Medical Genetics Laboratories, Baylor College of Medicine

Dr. Lee's BCM Department of Molecular and Human Genetics Faculty Page

Baylor College of Medicine Web Site

 

Contact Information:

Brendan Lee, M.D., Ph.D.
Principal Investigator
E-mail: blee@bcm.tmc.edu

Brendan Lanpher, MD
Research Fellow

Mary A. Mullins RN, BSN
Clinical Research Coordinator
Phone: (832) 822-4263
1-800-364-5437 extension 24263
E-mail: mullins@bcm.edu

 

Current Studies:

5101: Longitudinal Study of Urea Cycle Disorders

5102: A Randomized, Double-Blind, Crossover Study of Sodium Phenylbutyrate (BuphenylTM) and Low-Dose Arginine (100/mg/kg/day) Compared to High-Dose Arginine (500 mg/kg/day) Alone on Liver Function, Ureagenesis and Subsequent Nitric Oxide Production in Patients with Argininosuccinic Aciduria (ASA)

 

List of Publications:

  1. G. Patejunas, B. Lee, J.A. Dennis, P. Healy, P.J. Reeds, H. Yu, M. Frazer, B. Mull, A.W. Warman, A.L. Beaudet, and W.E. O'Brien, Evaluation of gene therapy for citrullinemia using murine and bovine models. Journal of Inherited Metabolic Disease, 21 (Supp1):138-150 (1998).
  2. B. Lee, J.A. Dennis, P.J. Healy, B. Mull, L. Pastore, H. Yu, E. Aguilar-Cordova, W. O'Brien, P. Reeds, and A.L. Beaudet, Hepatocyte gene therapy in a large animal, neonatal bovine model of citrullinemia. Proceedings of the National Academy of Sciences U.S.A., 96:39813986 (1999).
  3. B. Lee, H. Yu, F. Jahoor, W. O’Brien, A.L. Beaudet, and P. Reeds, In vivo urea cycle flux distinguishes and correlates with phenotypic severity in disorders of the urea cycle. Proceedings of the National Academy of Sciences U.S.A., 97:8021-8026 (2000).
  4. F. Scaglia, Q. Zheng, W. E. O'Brien, J. Henry, J. Rosenberger, P. Reeds, and B. Lee. An integrated approach to the diagnosis and prospective management of partial ornithine transcarbamylase deficiency. Pediatrics, 109:150-152 (2002).
  5. F. Scaglia, J. Rosenberger, J. Henry, B. Lee, and P. Reeds. Differential utilization of systemic and enteral ammonia for urea synthesis in control subjects and carriers for ornithine transcarbamylase deficiency. American Journal of Clinical Nutrition, 78:749-755 (2003).
  6. F. Scaglia, S. Carter, W. O’Brien, and B. Lee.  Effect of alternative pathway therapy on branched chain amino acid metabolism in urea cycle disorder patients. Molecular Genetics and Metabolism, 81S:79-85 (2004).
  7. B. Lee and J. Goss, Long-term correction of urea cycle disorders. Journal of Pediatrics, 138: S62S71 (2001).
  8. A. Mian and B. Lee. From molecular pathway to molecular therapy: Urea cycle disorders as a paradigm for inborn errors of hepatocyte metabolism, Trends in Molecular Medicine, 8:583-589 (2002).
  9. S. Kleppe, A. Mian, and B. Lee. Urea Cycle Disorders. Current Treatment Options in Neurology, 5:309-319 (2003).
  10. A. Mian, W.M. McCormack, V. Mane, S. Kleppe, P. Ng, M. Finegold, W.E. O’Brien, J.R. Rodgers, A.L. Beaudet, and B. Lee.; Long-term correction of ornithine transcarbamylase deficiency by WPRE-mediated over-expression using a helper-dependent adenovirus. Molecular Therapy, 10: 492-499 (2004).
  11. K. McBride, G. Miller, S. Carter, S. Karpen, J. Goss, and B. Lee. Developmental outcomes in early orthotopic liver transplantation for infants with neonatal onset urea cycle disorders and a female with late onset ornithine transcarbamylase deficiency. Pediatrics, 114:e523-526 (2004).
  12. F. Scaglia, N. Brunetti, S. Kleppe, J. Marini, S. Carter, P. Garlick, F. Jahoor, W. O’Brien, and B. Lee. Clinical Consequences of Urea Cycle Enzyme Deficiencies and Potential Links to Arginine and Nitric Oxide Metabolism. Journal of Nutrition, 2775S-2782S (2004).