M Yudkoff

Summary

Affiliation: The Children's Hospital of Philadelphia
Country: USA

Publications

  1. ncbi request reprint Agmatine stimulates hepatic fatty acid oxidation: a possible mechanism for up-regulation of ureagenesis
    Itzhak Nissim
    Children s Hospital of Philadelphia, Division of Child Development, Rehabilitation Medicine, and Metabolic Disease, Department of Pediatrics, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104, USA
    J Biol Chem 281:8486-96. 2006
  2. ncbi request reprint Short-term fasting, seizure control and brain amino acid metabolism
    Marc Yudkoff
    Department of Pediatrics, Children s Hospital of Philadelphia, University of Pennsylvania School of Medicine, 19104, USA
    Neurochem Int 48:650-6. 2006
  3. pmc Measuring in vivo ureagenesis with stable isotopes
    Marc Yudkoff
    Children s Hospital of Philadelphia, Department of Pediatrics, University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA
    Mol Genet Metab 100:S37-41. 2010
  4. ncbi request reprint Response of brain amino acid metabolism to ketosis
    Marc Yudkoff
    Department of Pediatrics, University of Pennsylvania School of Medicine, Children s Hospital of Philadelphia, 34th Street and Civic Center Boulevard, Philadelphia, PA 19104, USA
    Neurochem Int 47:119-28. 2005
  5. ncbi request reprint Ketogenic diet, brain glutamate metabolism and seizure control
    Marc Yudkoff
    Department of Pediatrics, University of Pennsylvania School of Medicine, Children s Hospital of Philadelphia, 34th Street and Civic Center Boulevard, Philadelphia, PA 19104, USA
    Prostaglandins Leukot Essent Fatty Acids 70:277-85. 2004
  6. ncbi request reprint Metabolism of brain amino acids following pentylenetetrazole treatment
    Marc Yudkoff
    Department of Pediatrics, Division of Child Development, School of Medicine, Children s Hospital of Philadelphia, University of Pennsylvania, 34th Street and Civic Center Blvd, Philadelphia, PA, USA
    Epilepsy Res 53:151-62. 2003
  7. ncbi request reprint The ketogenic diet and brain metabolism of amino acids: relationship to the anticonvulsant effect
    Marc Yudkoff
    Children s Hospital of Philadelphia and Department of Pediatrics, University of Pennsylvania School of Medicine, Philadelphia, PA, USA
    Annu Rev Nutr 27:415-30. 2007
  8. ncbi request reprint Mental Retardation Research Center: Children's Hospital of Philadelphia & University of Pennsylvania
    Marc Yudkoff
    Children s Hospital of Philadelphia, 1 Children s Center, Philadelphia, PA 19104 4318, USA
    Int J Dev Neurosci 20:439. 2002
  9. pmc Ketosis and brain handling of glutamate, glutamine, and GABA
    Marc Yudkoff
    Department of Pediatrics, Children s Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104, USA
    Epilepsia 49:73-5. 2008
  10. ncbi request reprint Acidosis and astrocyte amino acid metabolism
    M Yudkoff
    Children s Hospital of Philadelphia, Department of Pediatrics, University of Pennsylvania School of Medicine, 19104 4318, USA
    Neurochem Int 36:329-39. 2000

Research Grants

Collaborators

Detail Information

Publications38

  1. ncbi request reprint Agmatine stimulates hepatic fatty acid oxidation: a possible mechanism for up-regulation of ureagenesis
    Itzhak Nissim
    Children s Hospital of Philadelphia, Division of Child Development, Rehabilitation Medicine, and Metabolic Disease, Department of Pediatrics, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104, USA
    J Biol Chem 281:8486-96. 2006
    ..This action of agmatine may be mediated via a second messenger such as cAMP, and the effects on ureagenesis and fatty acid oxidation may occur simultaneously and/or independently...
  2. ncbi request reprint Short-term fasting, seizure control and brain amino acid metabolism
    Marc Yudkoff
    Department of Pediatrics, Children s Hospital of Philadelphia, University of Pennsylvania School of Medicine, 19104, USA
    Neurochem Int 48:650-6. 2006
    ....
  3. pmc Measuring in vivo ureagenesis with stable isotopes
    Marc Yudkoff
    Children s Hospital of Philadelphia, Department of Pediatrics, University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA
    Mol Genet Metab 100:S37-41. 2010
    ..Finally, the use of isotopes affords an ideal tool with which to gauge the efficacy of therapeutic interventions to augment residual flux through the cycle...
  4. ncbi request reprint Response of brain amino acid metabolism to ketosis
    Marc Yudkoff
    Department of Pediatrics, University of Pennsylvania School of Medicine, Children s Hospital of Philadelphia, 34th Street and Civic Center Boulevard, Philadelphia, PA 19104, USA
    Neurochem Int 47:119-28. 2005
    ....
  5. ncbi request reprint Ketogenic diet, brain glutamate metabolism and seizure control
    Marc Yudkoff
    Department of Pediatrics, University of Pennsylvania School of Medicine, Children s Hospital of Philadelphia, 34th Street and Civic Center Boulevard, Philadelphia, PA 19104, USA
    Prostaglandins Leukot Essent Fatty Acids 70:277-85. 2004
    ..Since brain glutamine is formed in astrocytes from glutamate, the overall effect will be to favor the release of glutamate from the nervous system...
  6. ncbi request reprint Metabolism of brain amino acids following pentylenetetrazole treatment
    Marc Yudkoff
    Department of Pediatrics, Division of Child Development, School of Medicine, Children s Hospital of Philadelphia, University of Pennsylvania, 34th Street and Civic Center Blvd, Philadelphia, PA, USA
    Epilepsy Res 53:151-62. 2003
    ..A net result of these processes may be to enable the brain more readily to dispose of the glutamate that is released from neurons during convulsive activity...
  7. ncbi request reprint The ketogenic diet and brain metabolism of amino acids: relationship to the anticonvulsant effect
    Marc Yudkoff
    Children s Hospital of Philadelphia and Department of Pediatrics, University of Pennsylvania School of Medicine, Philadelphia, PA, USA
    Annu Rev Nutr 27:415-30. 2007
    ..The ketogenic diet also may facilitate mechanisms by which the brain exports to blood compounds such as glutamine and alanine, in the process favoring the removal of glutamate carbon and nitrogen...
  8. ncbi request reprint Mental Retardation Research Center: Children's Hospital of Philadelphia & University of Pennsylvania
    Marc Yudkoff
    Children s Hospital of Philadelphia, 1 Children s Center, Philadelphia, PA 19104 4318, USA
    Int J Dev Neurosci 20:439. 2002
  9. pmc Ketosis and brain handling of glutamate, glutamine, and GABA
    Marc Yudkoff
    Department of Pediatrics, Children s Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104, USA
    Epilepsia 49:73-5. 2008
    ..This allows for: (a) more efficient removal of glutamate, the most important excitatory neurotransmitter; and (b) more efficient conversion of glutamine to GABA, the major inhibitory neurotransmitter...
  10. ncbi request reprint Acidosis and astrocyte amino acid metabolism
    M Yudkoff
    Children s Hospital of Philadelphia, Department of Pediatrics, University of Pennsylvania School of Medicine, 19104 4318, USA
    Neurochem Int 36:329-39. 2000
    ..The data point to the interplay of the metabolism of glucose and that of glutamate in these cells...
  11. ncbi request reprint Brain amino acid requirements and toxicity: the example of leucine
    Marc Yudkoff
    Children s Hospital of Philadelphia, Division of Child Development, Rehabilitation and Metabolic Disease, Department of Pediatrics, University of Pennsylvania School of Medicine, 19104, USA
    J Nutr 135:1531S-8S. 2005
    ..The result is a compromise of energy metabolism because of a failure of the malate-aspartate shuttle and a diminished rate of protein synthesis...
  12. ncbi request reprint Brain amino acid metabolism and ketosis
    M Yudkoff
    Division of Child Development and Rehabilitation, Children s Hospital of Philadelphia, 34th Street and Civic Center Boulevard, Philadelphia, PA 19104, USA
    J Neurosci Res 66:272-81. 2001
    ..e., ketone bodies, were precursor to aspartate, glutamate, glutamine and GABA...
  13. ncbi request reprint Ketogenic diet, amino acid metabolism, and seizure control
    M Yudkoff
    Division of Child Development and Rehabilitation, Children s Hospital of Philadelphia, 34th St and Civic Center Blvd, Philadelphia, PA 19104, USA
    J Neurosci Res 66:931-40. 2001
    ..We consider mechanisms by which such changes might lead to the antiepileptic effect...
  14. ncbi request reprint Evaluation of formulas for calculating total energy requirements of preadolescent children with cystic fibrosis
    Jillian Trabulsi
    Divisions of Gastroenterology, Hepatology, and Nutrition, The Children s Hospital of Philadelphia, Philadelphia, PA 19104, USA
    Am J Clin Nutr 85:144-51. 2007
    ..Care providers use one of several empirically derived formulas to calculate energy requirements, yet the validity of these formulas has seldom been tested...
  15. pmc In vivo nitrogen metabolism in ornithine transcarbamylase deficiency
    M Yudkoff
    Division of Child Development, Children s Hospital of Philadelphia, Pennsylvania 19104, USA
    J Clin Invest 98:2167-73. 1996
    ..g., liver transplantation and gene therapy...
  16. doi request reprint Very long-chain acyl-CoA dehydrogenase deficiency in a patient with normal newborn screening by tandem mass spectrometry
    Can Ficicioglu
    Department of Pediatrics, Section of Biochemical Genetics, The Children s Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA
    J Pediatr 156:492-4. 2010
    ..We report a patient who died as a result of severe brain injury due to hypoglycemia. Newborn screening was normal. Postmortem enzyme analysis and molecular testing confirmed the diagnosis of VLCADD...
  17. pmc Elimination of KATP channels in mouse islets results in elevated [U-13C]glucose metabolism, glutaminolysis, and pyruvate cycling but a decreased gamma-aminobutyric acid shunt
    Changhong Li
    Division of Endocrinology, The Children s Hospital of Philadelphia, University of Pennsylvania, Philadelphia, Pennsylvania 19104, USA
    J Biol Chem 283:17238-49. 2008
    ....
  18. ncbi request reprint Energy balance and the accuracy of reported energy intake in preadolescent children with cystic fibrosis
    Jillian Trabulsi
    Division of Gastroenterology, The Children s Hospital of Philadelphia, Department of Pediatrics, University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA
    Am J Clin Nutr 84:523-30. 2006
    ..Suboptimal growth and nutritional status are common among children with cystic fibrosis (CF) and pancreatic insufficiency (PI). A better understanding of energy balance is required to improve prevention and treatment of malnutrition...
  19. ncbi request reprint Botulinum toxin type a injections to salivary glands: combination with single event multilevel chemoneurolysis in 2 children with severe spastic quadriplegic cerebral palsy
    Heakyung Kim
    Department of Physical Medicine and Rehabilitation, University of Pennsylvania, School of Medicine, Philadelphia, PA, USA
    Arch Phys Med Rehabil 87:141-4. 2006
    ....
  20. pmc Role of the glutamate dehydrogenase reaction in furnishing aspartate nitrogen for urea synthesis: studies in perfused rat liver with 15N
    Itzhak Nissim
    Division of Child Development and Rehabilitation Medicine, Department of Pediatrics, Children s Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA
    Biochem J 376:179-88. 2003
    ..In addition, the current findings suggest that the formation of aspartate via the mitochondrial aspartate aminotransferase reaction may play an important role in the synthesis of cytosolic argininosuccinate...
  21. ncbi request reprint Regulation of urea synthesis by agmatine in the perfused liver: studies with 15N
    Itzhak Nissim
    Children s Hospital of Philadelphia and Division of Child Development and Rehabilitation, Department of Pediatrics, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104, USA
    Am J Physiol Endocrinol Metab 283:E1123-34. 2002
    ..These data suggest a possible therapeutic strategy encompassing the use of agmatine for the treatment of disturbed ureagenesis, whether secondary to inborn errors of metabolism or to liver disease...
  22. ncbi request reprint Effects of a GTP-insensitive mutation of glutamate dehydrogenase on insulin secretion in transgenic mice
    Changhong Li
    Division of Endocrinology, The Children s Hospital of Philadelphia and Diabetes Center, Philadelphia, Pennsylvania 19104, USA
    J Biol Chem 281:15064-72. 2006
    ..This study suggests that GDH functions predominantly in the direction of glutamate oxidation rather than glutamate synthesis in mouse islets and that this flux is tightly controlled by glucose...
  23. pmc Cross-sectional multicenter study of patients with urea cycle disorders in the United States
    Mendel Tuchman
    Children s National Medical Center, The George Washington University, School of Medicine, 111 Michigan Avenue, N W, Washington, DC 20010, USA
    Mol Genet Metab 94:397-402. 2008
    ..Plasma glutamine levels were higher in proximal UCD and in neonatal type disease. The RDCRN allows comprehensive analyses of rare inherited UCD, their frequencies and current medical practices...
  24. pmc Myc regulates a transcriptional program that stimulates mitochondrial glutaminolysis and leads to glutamine addiction
    David R Wise
    Department of Cancer Biology, Abramson Cancer Center, University of Pennsylvania, Philadelphia, PA 19104 6160, USA
    Proc Natl Acad Sci U S A 105:18782-7. 2008
    ..These data suggest that oncogenic levels of Myc induce a transcriptional program that promotes glutaminolysis and triggers cellular addiction to glutamine as a bioenergetic substrate...
  25. pmc Dynamic regulation of metabolism and respiration by endogenously produced nitric oxide protects against oxidative stress
    E Paxinou
    Stokes Research Institute, Children's Hospital of Philadelphia, University of Pennsylvania, Philadelphia, PA 19104, USA
    Proc Natl Acad Sci U S A 98:11575-80. 2001
    ....
  26. pmc Metabolic pathway profiling of mitochondrial respiratory chain mutants in C. elegans
    M J Falk
    Division of Human Genetics, Department of Pediatrics, The Children s Hospital of Philadelphia and University of Pennsylvania, Philadelphia, PA, USA
    Mol Genet Metab 93:388-97. 2008
    ..Such knowledge may enable the development of a metabolomic profiling diagnostic tool applicable to human mitochondrial disease...
  27. pmc Beyond aerobic glycolysis: transformed cells can engage in glutamine metabolism that exceeds the requirement for protein and nucleotide synthesis
    Ralph J DeBerardinis
    Department of Cancer Biology, Abramson Cancer Center, University of Pennsylvania, Room 450, BRB II III, 421 Curie Boulevard, Philadelphia, PA 19104 6160, USA
    Proc Natl Acad Sci U S A 104:19345-50. 2007
    ..Rather, glutamine metabolism provides a carbon source that facilitates the cell's ability to use glucose-derived carbon and TCA cycle intermediates as biosynthetic precursors...
  28. ncbi request reprint Regulation of leucine-stimulated insulin secretion and glutamine metabolism in isolated rat islets
    Changhong Li
    Division of Endocrinology, Children s Hospital of Philadelphia, Pennsylvania 19104, USA
    J Biol Chem 278:2853-8. 2003
    ..These mechanisms provide an explanation for hypoglycemia caused by mutations of GDH in children...
  29. ncbi request reprint A signaling role of glutamine in insulin secretion
    Changhong Li
    Division of Endocrinology, Children s Hospital of Philadelphia, Philadelphia, Pennsylvania 19104, USA
    J Biol Chem 279:13393-401. 2004
    ....
  30. ncbi request reprint The sparse fur mouse as a model for gene therapy in ornithine carbamoyltransferase deficiency
    M L Batshaw
    Department of Pediatrics, University of Pennsylvania School of Medicine, Philadelphia 19104, USA
    Gene Ther 2:743-9. 1995
    ..These clearly definable metabolic and behavioral abnormalities suggest that the spf/Y mouse should prove a useful model for studying the efficacy of gene therapy in OCTD...
  31. ncbi request reprint A pilot study of in vivo liver-directed gene transfer with an adenoviral vector in partial ornithine transcarbamylase deficiency
    Steven E Raper
    Institute for Human Gene Therapy and Department of Surgery, University of Pennsylvania, Philadelphia, PA 19104, USA
    Hum Gene Ther 13:163-75. 2002
    ..The low levels of gene transfer detected in this trial suggest that at the doses tested, significant metabolic correction did not occur...
  32. pmc Complex management of a patient with a contiguous Xp11.4 gene deletion involving ornithine transcarbamylase: a role for detailed molecular analysis in complex presentations of classical diseases
    Matthew A Deardorff
    Division of Metabolic Diseases, The Children s Hospital of Philadelphia, Philadelphia, PA, USA
    Mol Genet Metab 94:498-502. 2008
    ....
  33. pmc 3-isobutylmethylxanthine inhibits hepatic urea synthesis: protection by agmatine
    Itzhak Nissim
    Children s Hospital of Philadelphia, Division of Child Development, Department of Pediatrics, University of Pennsylvania School of Medicine, 34th Street and Civic Center Boulevard, Philadelphia, PA 19104, USA
    J Biol Chem 283:15063-71. 2008
    ..Together, the data may suggest a new experimental application of IBMX in studies of CPS-I malfunction and the use of agmatine as intervention therapy...
  34. ncbi request reprint The role of mitochondrially bound arginase in the regulation of urea synthesis: studies with [U-15N4]arginine, isolated mitochondria, and perfused rat liver
    Itzhak Nissim
    Children s Hospital of Philadelphia, Division of Child Development and Rehabilitation Medicine, Department of Pediatrics, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104, USA
    J Biol Chem 280:17715-24. 2005
    ....
  35. pmc N-carbamylglutamate markedly enhances ureagenesis in N-acetylglutamate deficiency and propionic acidemia as measured by isotopic incorporation and blood biomarkers
    Mendel Tuchman
    Children s Research Institute, Children s National Medical Center, George Washington University, Washington, DC 20052, USA
    Pediatr Res 64:213-7. 2008
    ..These results provide a reliable method for measuring the effect of NCG on nitrogen metabolism and strongly suggest that NCG could be an effective treatment for inherited and secondary NAGS deficiency...
  36. pmc 1H MRS identifies symptomatic and asymptomatic subjects with partial ornithine transcarbamylase deficiency
    A L Gropman
    Department of Neurology, Children s National Medical Center, George Washington University School of Medicine and Health Sciences, Washington, DC 20010, USA
    Mol Genet Metab 95:21-30. 2008
    ..To evaluate brain metabolism in subjects with partial ornithine transcarbamylase deficiency (OTCD) utilizing (1)H MRS...
  37. pmc 1H MRS allows brain phenotype differentiation in sisters with late onset ornithine transcarbamylase deficiency (OTCD) and discordant clinical presentations
    Andrea L Gropman
    Department of Neurology, Children s National Medical Center, 111 Michigan Avenue, NW, Washington, DC 20010, USA
    Mol Genet Metab 94:52-60. 2008
    ..The concentration of mI seen on (1)H MRS in PWM and FWM in this family could be used to deduce clinical symptomatology and may serve as a non-invasive marker of brain liability in OTCD...
  38. ncbi request reprint Restoration of ureagenesis in N-acetylglutamate synthase deficiency by N-carbamylglutamate
    Ljubica Caldovic
    Children s Research Institute, Children s National Medical Center, The George Washington University, Washington, DC 20010, USA
    J Pediatr 145:552-4. 2004
    ..Blood ammonia rose following ammonium tracer ingestion before treatment but remained low following treatment. Serum urea concentration doubled following the treatment...

Research Grants7

  1. KETOGENIC DIET AND BRAIN AMINO ACID METABOLISM
    Marc Yudkoff; Fiscal Year: 2002
    ....
  2. KETOGENIC DIET AND BRAIN AMINO ACID METABOLISM
    Marc Yudkoff; Fiscal Year: 2001
    ....
  3. Mental Retardation and Development Disabilities Research
    Marc Yudkoff; Fiscal Year: 2007
    ..The Center includes more than 60 Penn faculty members representing 15 departments at the School of Medicine, School of Veterinary Medicine, The School of Nursing, the Wistar Institute, and the College of Arts and Sciences. ..