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- W2017358216 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-alpha (TNF-alpha) bioactivity, but not TNF-alpha 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-alpha or levels of soluble TNF receptor did not account for bioactivity differences or phenotypic heterogeneity in X-ALD." @default.
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- W2017358216 date "1995-09-01" @default.
- W2017358216 modified "2023-10-14" @default.
- W2017358216 title "Tumor necrosis factor-α and X-linked adrenoleukodystrophy" @default.
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- W2017358216 doi "https://doi.org/10.1016/0165-5728(95)00084-f" @default.
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