Tumor necrosis factor-α and X-linked adrenoleukodystrophy

M. C. Mcguinness, D. E. Griffin, G. V. Raymond, C. A. Washington, H. W. Moser, K. D. Smith

Research output: Contribution to journalArticlepeer-review

47 Scopus citations

Abstract

The two most common forms of X-linked adrenoleukodystrophy (X-ALD), the childhood cerebral form (CCER) and the adult form, adrenomyeloneuropathy (AMN), arise from the same mutations in the X-ALD gene at Xq28. These two forms are distinguished by the degree of cerebral inflammation. Segregation analysis suggests that an autosomal modifying gene may be a major determinant of phenotype in X-ALD. Thus, a modifying gene could be involved in initiating or promoting the inflammatory response. In this study we detected a difference in tumor necrosis factor-α (TNF-α) bioactivity, but not TNF-α protein levels, in serum from some advanced CCER patients. Early-stage CCER patients and AMN patients were in the normal range. Allelic differences in TNF-α or levels of soluble TNF receptor did not account for bioactivity differences or phenotypic heterogeneity in X-ALD.

Original languageEnglish (US)
Pages (from-to)161-169
Number of pages9
JournalJournal of Neuroimmunology
Volume61
Issue number2
DOIs
StatePublished - Sep 1995

Keywords

  • Adrenoleukodystrophy
  • Adrenomyeloneuropathy
  • Tumor necrosis factor-α

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