Immunocytochemical investigations of some human leukodystrophies.
One case of each of the following human leukodystrophies was examined immunocytochemically with antisera against myelin basic protein (MBP), myelin associated glycoprotein (MAG) and gliofibrillary acidic protein (GFA): Metachromatic leukodystrophy (MLD), connatal adrenoleukodystrophy (ALD), sudanophilic leukodystrophy of the adult (SLD) and connatal Pelizaeus-Merzbacher disease (PMD). A case of canine globoid cell leukodystrophy (GLD) was also included under the assumption that this disease was the same in the dog as in man. It was shown that the storage process in MLD and GLD did not involve MBP or MAG and that the breakdown of myelin with the formation of fat granule cells containing droplets of neutral fat in ALD and SLS proceeds in a similar way as in experimental Wallerian degeneration. In PMD, MBP is present in the vicinity of axons not surrounded by a myelin sheath demonstrable with conventional means. The globoid cells of GLD could be demonstrated to be of non-astrocytic origin.
D000293:Adolescent; D000326:Adrenoleukodystrophy / Q000378:metabolism; D000818:Animals; D002675:Child, Preschool; D002549:Diffuse Cerebral Sclerosis of Schilder / Q000378:metabolism*; D004285:Dogs; D005260:Female; D005904:Glial Fibrillary Acidic Protein; D006801:Humans; D007381:Intermediate Filament Proteins / Q000032:analysis; D007965:Leukodystrophy, Globoid Cell / Q000378:metabolism; D007966:Leukodystrophy, Metachromatic / Q000378:metabolism; D008297:Male; D008875:Middle Aged; D004676:Myelin Basic Protein / Q000032:analysis; D009185:Myelin Proteins / Q000032:analysis; D018992:Myelin-Associated Glycoprotein
D005904:Glial Fibrillary Acidic Protein; D007381:Intermediate Filament Proteins; D004676:Myelin Basic Protein; D009185:Myelin Proteins; D018992:Myelin-Associated Glycoprotein