HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS		QUICK SEARCH:   [advanced] Author: Keyword(s): Year:  Vol:  Page:

This Article Full Text (PDF) Correspondence: Submit a response Alert me when this article is cited Alert me when Correspondence are posted Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Rizzo, W. B. Articles by Schulman, J. D. Search for Related Content PubMed PubMed Citation Articles by Rizzo, W. B. Articles by Schulman, J. D.

Adrenoleukodystrophy

Very long-chain fatty acid metabolism in fibroblasts

From the Section on Biochemical Genetics, National Institute of Child Health and Human Development (Drs. Rizzo, Chemke, and Schulman), and the Laboratory of Cellular Metabolism, National Heart, Lung and Blood Institute (Dr. Avigan), NIH, Bethesda, MD.

We studied very long-chain fatty acid (VLFA) metabolism in cultured fibroblasts from patients with adrenoleukodystrophy (ALD). Total hexacosanoate (C26:0) content of ALD fibroblasts was sixfold higher than normal and did not return to normal when cells were grown in lipid-free medium. When normal or ALD fibroblasts were grown in medium containing 10% ALD serum (which is enriched in C26:0), there was no further increase in C26:0 content compared with cells grown in 10% normal human serum. Uptake and loss of 1-¹⁴C-palmitate (C16:0) and 1-¹⁴C-lignocerate (C24:0) by ALD fibroblasts were similar to normal fibroblasts. Catabolism of exogenous H-C26:0 to H₂O was about 307 of normal. Oxidation of exogenous 1-¹⁴C-hexacosanoate, 1-¹⁴C-lignocerate, and 1-¹⁴C-palmitate in intact ALD fibroblasts was 42%, 27 ± 13% (SD), and 73 ± 47%, respectively, of normal. These results are consistent with, but do not conclusively prove, a VLFA oxidation defect in ALD fibroblasts.

Address correspondence and reprint requests to Dr. Rizzo, Department of Pediatrics, Medical College of Virginia, P.O. Box 2.59, MCV Station, Richmond, VA 23298.

Accepted for publication May 23, 1983.

This article has been cited by other articles:

				A. Brolinson, S. Fourcade, A. Jakobsson, A. Pujol, and A. Jacobsson Steroid Hormones Control Circadian Elovl3 Expression in Mouse Liver Endocrinology, June 1, 2008; 149(6): 3158 - 3166. [Abstract] [Full Text] [PDF]

				M. Asheuer, I. Bieche, I. Laurendeau, A. Moser, B. Hainque, M. Vidaud, and P. Aubourg Decreased expression of ABCD4 and BG1 genes early in the pathogenesis of X-linked adrenoleukodystrophy Hum. Mol. Genet., May 15, 2005; 14(10): 1293 - 1303. [Abstract] [Full Text] [PDF]

				T. J. Ferraro Lorenzo's Chrism South Atlantic Quarterly, January 1, 2004; 103(1): 235 - 263. [PDF]

				A. K. Heinzer, P. A. Watkins, J.-F. Lu, S. Kemp, A. B. Moser, Y. Y. Li, S. Mihalik, J. M. Powers, and K. D. Smith A very long-chain acyl-CoA synthetase-deficient mouse and its relevance to X-linked adrenoleukodystrophy Hum. Mol. Genet., May 15, 2003; 12(10): 1145 - 1154. [Abstract] [Full Text] [PDF]

				A. K. Heinzer, S. Kemp, J.-F. Lu, P. A. Watkins, and K. D. Smith Mouse Very Long-chain Acyl-CoA Synthetase in X-linked Adrenoleukodystrophy J. Biol. Chem., August 2, 2002; 277(32): 28765 - 28773. [Abstract] [Full Text] [PDF]

				J.-F. Lu, A. M. Lawler, P. A. Watkins, J. M. Powers, A. B. Moser, H. W. Moser, and K. D. Smith A mouse model for X-linked adrenoleukodystrophy PNAS, August 19, 1997; 94(17): 9366 - 9371. [Abstract] [Full Text] [PDF]

				A. K. Percy Review Article: The Inherited Neurodegenerative Disorders of Childhood: Clinical Assessment J Child Neurol, April 1, 1987; 2(2): 82 - 97. [Abstract] [PDF]