A comparative study of myelin fractions from metachromatic and globoid leukodystrophies.
Myelin was isolated post mortem from brains of patients with metachromatic and globoid forms of leukodystrophy. In the leukodystrophies, isolates were obtained from central white matter and subcortical "U" fiber areas. Myelin also was obtained from age-matched human controls, and the method yielded light and heavy myelin fractions. In both disorders, the pathologic process affected the heavy isolates to a relatively greater degree. Comparative chemical analyses were made of the myelin isolates, and we concluded that a similar pathogenesis--the production of an unstable membrane in later stages of myelin formation--affects both disorders.
D002554:Cerebrosides / Q000032:analysis; D002784:Cholesterol / Q000032:analysis; D006017:Glycolipids / Q000032:analysis; D006801:Humans; D007223:Infant; D007965:Leukodystrophy, Globoid Cell / Q000378:metabolism*; D007966:Leukodystrophy, Metachromatic / Q000378:metabolism*; D009186:Myelin Sheath / Q000032:analysis*; D009419:Nerve Tissue Proteins / Q000032:analysis; D010743:Phospholipids / Q000032:analysis; D013433:Sulfoglycosphingolipids / Q000032:analysis
D002554:Cerebrosides; D006017:Glycolipids; D009419:Nerve Tissue Proteins; D010743:Phospholipids; D013433:Sulfoglycosphingolipids; D002784:Cholesterol