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Human Molecular Genetics, 2001, Vol. 10, No. 19 2069-2077
© 2001 Oxford University Press

Gestational, pathologic and biochemical differences between very long-chain acyl-CoA dehydrogenase deficiency and long-chain acyl-CoA dehydrogenase deficiency in the mouse

Keith B. Cox, Doug A. Hamm, David S. Millington1, Dietrich Matern2, Jerry Vockley3, Piero Rinaldo2, Carl A. Pinkert, William J. Rhead4, J. Russell Lindsey and Philip A. Wood+

Department of Genomics and Pathobiology, 1670 University Boulevard, University of Alabama at Birmingham, Birmingham, AL 35294-0019, USA, 1Mass Spectrometry Facility, Duke University, Research Triangle Park, NC 27709, USA, 2Department of Laboratory Medicine and Pathology and 3Department of Medical Genetics, Mayo Clinic, Rochester, MN 55905, USA and 4Department of Pediatrics, University of Iowa, Iowa City, IA 52242, USA

Although many patients have been found to have very long-chain acyl-CoA dehydrogenase (VLCAD) deficiency, none have been documented with long-chain acyl-CoA dehydrogenase (LCAD) deficiency. In order to understand the metabolic pathogenesis of long-chain fatty acid oxidation disorders, we generated mice with VLCAD deficiency (VLCAD–/–) and compared their pathologic and biochemical phenotypes of mice with LCAD deficiency (LCAD–/–) and wild-type mice. VLCAD–/– mice had milder fatty change in liver and heart. Dehydrogenation of various acyl-CoA substrates by liver, heart and skeletal muscle mitochondria differed among the three genotypes. The results for liver were most informative as VLCAD–/– mice had a reduction in activity toward palmitoyl-CoA and oleoyl-CoA (58 and 64% of wild-type, respectively), whereas LCAD–/– mice showed a more profoundly reduced activity toward these substrates (35 and 32% of wild-type, respectively), with a significant reduction of activity toward the branched chain substrate 2,6-dimethylheptanoyl-CoA. C16 and C18 acylcarnitines were elevated in bile, blood and serum of fasted VLCAD–/– mice, whereas abnormally elevated C12 and C14 acylcarnitines were prominent in LCAD–/– mice. Progeny with the combined LCAD+/+//VLCAD+/– genotype were over-represented in offspring from sires and dams heterozygous for both LCAD and VLCAD mutations. In contrast, no live mice with a compound LCAD–/–//VLCAD–/– genotype were detected.

+ To whom correspondence should be addressed. Tel: +1 205 934 1303; Fax: +1 205 975 4418; Email: paw@uab.edu


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