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- Publications
- Influence
Treatment of cardiomyopathy and rhabdomyolysis in long-chain fat oxidation disorders using an anaplerotic odd-chain triglyceride.
- C. Roe, L. Sweetman, D. Roe, F. David, H. Brunengraber
- Medicine, Biology
- The Journal of clinical investigation
- 15 July 2002
The current dietary treatment of long-chain fatty acid oxidation defects (high carbohydrate with medium-even-chain triglycerides and reduced amounts of long-chain fats) fails, in many cases, to… Expand
Anticonvulsant effects of a triheptanoin diet in two mouse chronic seizure models
- S. Willis, J. Stoll, L. Sweetman, Karin Borges
- Biology, Medicine
- Neurobiology of Disease
- 1 December 2010
We hypothesized that in epileptic brains citric acid cycle intermediate levels may be deficient leading to hyperexcitability. Anaplerosis is the metabolic refilling of deficient metabolites. Our goal… Expand
Increased anion gap metabolic acidosis as a result of 5-oxoproline (pyroglutamic acid): a role for acetaminophen.
- A. Fenves, H. Kirkpatrick, Viralkumar Patel, L. Sweetman, M. Emmett
- Medicine
- Clinical journal of the American Society of…
- 1 May 2006
The endogenous organic acid metabolic acidoses that occur commonly in adults include lactic acidosis; ketoacidosis; acidosis that results from the ingestion of toxic substances such as methanol,… Expand
Dietary anaplerotic therapy improves peripheral tissue energy metabolism in patients with Huntington's disease
We previously identified a systemic metabolic defect associated with early weight loss in patients with Huntington's disease (HD), suggesting a lack of substrates for the Krebs cycle. Dietary… Expand
Inhibition by propionyl-coenzyme A of N-acetylglutamate synthetase in rat liver mitochondria. A possible explanation for hyperammonemia in propionic and methylmalonic acidemia.
- F. X. Coudé, L. Sweetman, W. Nyhan
- Chemistry, Medicine
- The Journal of clinical investigation
- 1 December 1979
In the search for the mechanism by which hyperammonemia complicates propionic and methylmalonic acidemia the effects of a series of acyl-coenzyme A (CoA) derivatives were studied on the activity of… Expand
Abnormal metabolites of isoleucine in a patient with propionyl-CoA carboxylase deficiency.
- L. Sweetman, W. Weyler, W. Nyhan, C. de Céspedes, A. R. Loria, Y. Estrada
- Chemistry, Medicine
- Biomedical mass spectrometry
- 1 March 1978
A number of previously unrecognized abnormal metabolites have been identified and quantitated in the urine of a patient with an inherited deficiency of propionyl-CoA carboxylase. These included the… Expand
Short-chain acyl-coenzyme A dehydrogenase deficiency. Clinical and biochemical studies in two patients.
- B. Amendt, C. Greene, +5 authors W. Rhead
- Medicine, Biology
- The Journal of clinical investigation
- 1 May 1987
We describe two patients with short-chain acyl-coenzyme A (CoA) dehydrogenase (SCADH) deficiency. Neonate I excreted large amounts of ethylmalonate and methylsuccinate; ethylmalonate excretion… Expand
Biotin-response organicaciduria. Multiple carboxylase defects and complementation studies with propionicacidemia in cultured fibroblasts.
- M. Saunders, L. Sweetman, B. Robinson, K. Roth, R. Cohn, R. Gravel
- Biology, Medicine
- The Journal of clinical investigation
- 1 December 1979
Fibroblast cultures from two individuals with biotin-responsive organicacidemia were found to have a pleiotropic deficiency of propionyl-CoA carboxylase, beta-methylcrotonyl-CoA carboxylase, and… Expand
Multiple carboxylase deficiency: clinical and biochemical improvement following neonatal biotin treatment.
Multiple carboxylase deficiency is characterized by deficient activities of three biotin-dependent enzymes, propionyl coenzyme A carboxylase, pyruvate carboxylase, and beta-methylcrotonyl coenzyme A… Expand