TY - JOUR
T1 - Tumor necrosis factor-α and X-linked adrenoleukodystrophy
AU - Mcguinness, M. C.
AU - Griffin, D. E.
AU - Raymond, G. V.
AU - Washington, C. A.
AU - Moser, H. W.
AU - Smith, K. D.
PY - 1995/9
Y1 - 1995/9
N2 - 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.
AB - 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.
KW - Adrenoleukodystrophy
KW - Adrenomyeloneuropathy
KW - Tumor necrosis factor-α
UR - http://www.scopus.com/inward/record.url?scp=0028973256&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=0028973256&partnerID=8YFLogxK
U2 - 10.1016/0165-5728(95)00084-F
DO - 10.1016/0165-5728(95)00084-F
M3 - Article
C2 - 7593551
AN - SCOPUS:0028973256
SN - 0165-5728
VL - 61
SP - 161
EP - 169
JO - Journal of Neuroimmunology
JF - Journal of Neuroimmunology
IS - 2
ER -