A Defect in the Transport of Long-Chain Fatty Acids Associated with Acute Liver Failure
Ali Al Odaib, M.S., Benjamin L. Shneider, M.D., Michael J. Bennett, Ph.D., F.R.C.Path., Barbara R. Pober, M.D., Miguel Reyes-Mugica, M.D., Amy L. Friedman, M.D., Frederick J. Suchy, M.D., and Piero Rinaldo, M.D., Ph.D.
Since this article has no abstract, we have provided an extract of the first 100 words of the full text and any section headings.
Fatty-acid oxidation has a major role in energy production duringperiods of fasting. When body glucose is depleted, fatty acidsare mobilized from adipose tissue, taken up by the liver, andconverted to ketone bodies, a major alternative source of energyfor peripheral tissues.1 At the cellular level, after beingtransported through the cell membrane and then into the mitochondriaby means of a carnitine-dependentsystem, long-chain fatty acidsare predominantly oxidized in mitochondria.2,3
Common clinical features of disorders of fatty-acid oxidationare metabolic decompensation during fasting, hypoketotic hypoglycemia,and acute dysfunction of fatty-acid–dependent tissues(skeletal muscle, heart, and liver), . . . [Full Text of this Article]
Case Reports
Patient 1
Patient 2
Methods
Biochemical and Histologic Studies
Cell Lines and Enzyme Assays
Results
Discussion
Source Information
From the Departments of Genetics (A.A.O., B.R.P., P.R.), Pediatrics (B.L.S., F.J.S.), Pathology (M.R.-M.), and Surgery (A.L.F.), Yale University School of Medicine, New Haven, Conn.; and the Departments of Pathology and Pediatrics, University of Texas Southwestern Medical Center, Dallas (M.J.B.).
Address reprint requests to Dr. Rinaldo at the Department of Laboratory Medicine and Pathology, Division of Laboratory Genetics, Mayo Clinic, 200 First St. SW, Rochester, MN 55905.
References
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