Increment of alpha B-crystallin mRNA in the brain of patient with infantile type Alexander's disease

To estimate the expression level of alpha B-crystallin in the brain of infantile type Alexander's disease, the amounts of protein and mRNA of alpha B-crystallin were measured by enzyme immunoassay (EIA) and Northern blot analysis, respectively, in the brain of patient and controls, and in the tissues from glioblastoma and astrocytoma. The alpha B-crystallin protein in the brain of patient was remarkably increased as compared with those of controls. The amount of alpha B-crystallin mRNA of patient was increased about 7-fold compared to the mean value of the control group and higher than that of glioblastoma tissue. These data suggest that increment of alpha B-crystallin mRNA in astrocytes leads to the overexpression of this protein and may be one of the main causes of infantile type Alexander's disease.