Adrenoleukodystrophy: Very long-chain fatty acid metabolism in fibroblasts

William B. Rizzo, Joel Avigan, Juan Chemke, Joseph D. Schulman

Research output: Contribution to journalArticle

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Abstract

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 3H-C26:0 to 3H14O was about 30% 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.

Original languageEnglish (US)
Pages (from-to)163-169
Number of pages7
JournalNeurology
Volume34
Issue number2
StatePublished - Feb 1984

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Adrenoleukodystrophy
Fatty Acids
Fibroblasts
Palmitates
Serum
Lipids

ASJC Scopus subject areas

  • Clinical Neurology

Cite this

Rizzo, W. B., Avigan, J., Chemke, J., & Schulman, J. D. (1984). Adrenoleukodystrophy: Very long-chain fatty acid metabolism in fibroblasts. Neurology, 34(2), 163-169.

Adrenoleukodystrophy : Very long-chain fatty acid metabolism in fibroblasts. / Rizzo, William B.; Avigan, Joel; Chemke, Juan; Schulman, Joseph D.

In: Neurology, Vol. 34, No. 2, 02.1984, p. 163-169.

Research output: Contribution to journalArticle

Rizzo, WB, Avigan, J, Chemke, J & Schulman, JD 1984, 'Adrenoleukodystrophy: Very long-chain fatty acid metabolism in fibroblasts', Neurology, vol. 34, no. 2, pp. 163-169.
Rizzo WB, Avigan J, Chemke J, Schulman JD. Adrenoleukodystrophy: Very long-chain fatty acid metabolism in fibroblasts. Neurology. 1984 Feb;34(2):163-169.
Rizzo, William B. ; Avigan, Joel ; Chemke, Juan ; Schulman, Joseph D. / Adrenoleukodystrophy : Very long-chain fatty acid metabolism in fibroblasts. In: Neurology. 1984 ; Vol. 34, No. 2. pp. 163-169.
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