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4 in the algorithm for multiple sequence alignment. Surgical ligation of a patent ductus arteriosus. Circulation 2002;106:I241247. Accounts to. OH HBr HBr OH2 Br 8087; ca. 5) of the solution to be examined. 52, AR or X-linked AR AR X-linked AR AR AR Genetic lesion Peroxisomal membrane protein in the ABC transporter family Aspartoacylase PMP-22, P0, connexin-32 and other genes Galactocerebroside- β-galactosidase Aryl sulfatase A PLP Phenylalanine hydroxylase Oxidation of branched chain fatty acids; phytan-olyl CoA 2-hydroxylase in some cases Eukaryotic initiation factor 2B (eIF2B) Comments Decreased peroxidation of saturated, very-long- chain fatty acids, causing their accumulation in brain, adrenals and other tissues; variable phenotypes with regard to hypomyelination; see text Widespread white matter edema with diminished myelin; N-acetylaspartic aciduria; see text Variable degrees of myelin deficiency specific for the PNS; see text Globoid cells contain galactocerebroside; see text Accumulation of sulfatide in brain; see text Variable hypomyelination due to different mutations in the major structural protein of CNS myelin; similar to rodent mutants such as the jimpy mouse White matter is up to 40 deficient in myelin; hypomyelination may be caused by inhibition of amino acid transport andor protein synthesis by the high level of phenylalanine that accumulates Increase of branched-chain phytanic acid, especially in PNS myelin Also referred to as childhood ataxia with central hypomyelination (CACH); see text References 1, 26, Ch.
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