Marc Yudkoff

Department of Pediatrics, The Children's Hospital of Philadelphia

Affiliated withThe Children's Hospital of Philadelphia University of Pennsylvania

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JoVE Journal Publications

ArticleTotal : 1	Year
Publication title Cited by 12	2011

Other Publications

Article	Year
A pilot study of in vivo liver-directed gene transfer with an adenoviral vector in partial ornithine transcarbamylase deficiency. Human gene therapy\| PubMed ID: 11779420	2002
Mental Retardation Research Center: Children's Hospital of Philadelphia & University of Pennsylvania. International journal of developmental neuroscience : the official journal of the International Society for Developmental Neuroscience\| PubMed ID: 12175883	2002
Regulation of urea synthesis by agmatine in the perfused liver: studies with 15N. American journal of physiology. Endocrinology and metabolism\| PubMed ID: 12388162	2002
Regulation of leucine-stimulated insulin secretion and glutamine metabolism in isolated rat islets. The Journal of biological chemistry\| PubMed ID: 12444083	2003
Metabolism of brain amino acids following pentylenetetrazole treatment. Epilepsy research\| PubMed ID: 12576176	2003
Role of the glutamate dehydrogenase reaction in furnishing aspartate nitrogen for urea synthesis: studies in perfused rat liver with 15N. The Biochemical journal\| PubMed ID: 12935293	2003
A signaling role of glutamine in insulin secretion. The Journal of biological chemistry\| PubMed ID: 14736887	2004
Ketogenic diet, brain glutamate metabolism and seizure control. Prostaglandins, leukotrienes, and essential fatty acids\| PubMed ID: 14769486	2004
Restoration of ureagenesis in N-acetylglutamate synthase deficiency by N-carbamylglutamate. The Journal of pediatrics\| PubMed ID: 15480384	2004
Biosynthesis of agmatine in isolated mitochondria and perfused rat liver: studies with 15N-labelled arginine. The Biochemical journal\| PubMed ID: 15656789	2005
The role of mitochondrially bound arginase in the regulation of urea synthesis: studies with [U-15N4]arginine, isolated mitochondria, and perfused rat liver. The Journal of biological chemistry\| PubMed ID: 15753084	2005
Response of brain amino acid metabolism to ketosis. Neurochemistry international\| PubMed ID: 15888376	2005
Brain amino acid requirements and toxicity: the example of leucine. The Journal of nutrition\| PubMed ID: 15930465	2005
Botulinum toxin type a injections to salivary glands: combination with single event multilevel chemoneurolysis in 2 children with severe spastic quadriplegic cerebral palsy. Archives of physical medicine and rehabilitation\| PubMed ID: 16401453	2006
Agmatine stimulates hepatic fatty acid oxidation: a possible mechanism for up-regulation of ureagenesis. The Journal of biological chemistry\| PubMed ID: 16452488	2006
Short-term fasting, seizure control and brain amino acid metabolism. Neurochemistry international\| PubMed ID: 16510212	2006
Effects of a GTP-insensitive mutation of glutamate dehydrogenase on insulin secretion in transgenic mice. The Journal of biological chemistry\| PubMed ID: 16574664	2006
Ifosfamide-induced nephrotoxicity: mechanism and prevention. Cancer research\| PubMed ID: 16885387	2006
Energy balance and the accuracy of reported energy intake in preadolescent children with cystic fibrosis. The American journal of clinical nutrition\| PubMed ID: 16960165	2006
Evaluation of formulas for calculating total energy requirements of preadolescent children with cystic fibrosis. The American journal of clinical nutrition\| PubMed ID: 17209190	2007
The ketogenic diet and brain metabolism of amino acids: relationship to the anticonvulsant effect. Annual review of nutrition\| PubMed ID: 17444813	2007
Beyond aerobic glycolysis: transformed cells can engage in glutamine metabolism that exceeds the requirement for protein and nucleotide synthesis. Proceedings of the National Academy of Sciences of the United States of America\| PubMed ID: 18032601	2007
1H MRS allows brain phenotype differentiation in sisters with late onset ornithine transcarbamylase deficiency (OTCD) and discordant clinical presentations. Molecular genetics and metabolism\| PubMed ID: 18262815	2008
3-isobutylmethylxanthine inhibits hepatic urea synthesis: protection by agmatine. The Journal of biological chemistry\| PubMed ID: 18375388	2008
N-carbamylglutamate markedly enhances ureagenesis in N-acetylglutamate deficiency and propionic acidemia as measured by isotopic incorporation and blood biomarkers. Pediatric research\| PubMed ID: 18414145	2008
Primary coenzyme Q deficiency in Pdss2 mutant mice causes isolated renal disease. PLoS genetics\| PubMed ID: 18437205	2008
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. The Journal of biological chemistry\| PubMed ID: 18445600	2008
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 dise... Molecular genetics and metabolism\| PubMed ID: 18524659	2008
Cross-sectional multicenter study of patients with urea cycle disorders in the United States. Molecular genetics and metabolism\| PubMed ID: 18562231	2008
Myc regulates a transcriptional program that stimulates mitochondrial glutaminolysis and leads to glutamine addiction. Proceedings of the National Academy of Sciences of the United States of America\| PubMed ID: 19033189	2008
Ketosis and brain handling of glutamate, glutamine, and GABA. Epilepsia\| PubMed ID: 19049594	2008
Effects of a single dose of N-carbamylglutamate on the rate of ureagenesis. Molecular genetics and metabolism\| PubMed ID: 19660971	2009
Very long-chain acyl-CoA dehydrogenase deficiency in a patient with normal newborn screening by tandem mass spectrometry. The Journal of pediatrics\| PubMed ID: 20056241	2010
Inflammation-induced preterm birth alters neuronal morphology in the mouse fetal brain. Journal of neuroscience research\| PubMed ID: 20155801	2010
Establishing a consortium for the study of rare diseases: The Urea Cycle Disorders Consortium. Molecular genetics and metabolism\| PubMed ID: 20188616	2010
N-acetylglutamate synthase: structure, function and defects. Molecular genetics and metabolism\| PubMed ID: 20303810	2010
Measuring in vivo ureagenesis with stable isotopes. Molecular genetics and metabolism\| PubMed ID: 20338795	2010
N-carbamylglutamate augments ureagenesis and reduces ammonia and glutamine in propionic acidemia. Pediatrics\| PubMed ID: 20566609	2010
Down-regulation of hepatic urea synthesis by oxypurines: xanthine and uric acid inhibit N-acetylglutamate synthase. The Journal of biological chemistry\| PubMed ID: 21540182	2011
N-carbamylglutamate enhancement of ureagenesis leads to discovery of a novel deleterious mutation in a newly defined enhancer of the NAGS gene and to effective therapy. Human mutation\| PubMed ID: 21681857	2011
The glutamate transporter, GLAST, participates in a macromolecular complex that supports glutamate metabolism. Neurochemistry international\| PubMed ID: 22306776	2012