Abstract
Spongy degeneration (SD) of Van Bogaert and Bertrand in infancy is characterized by white matter edema and splitting of myelin at the major dense line. The cause of myelin breakdown in this disorder is believed to be secondary to the edema. Na, K ATPase activity was studied in 3 SD brains since inhibition of this enzyme is associated with edema. Na, K ATPase activity in homogenates from frontal cortex was reduced 35-60% in SD brains compared to leukodystrophic and non-neurological controls. The decreased enzymatic activity in SD was not due to altered affinity for either the activating cations Na and K or for the substrate ATP. Measurements of ouabain binding indicated that SD brain had approximately 2X the number of enzyme sites per mg membrane protein as compared with controls. The ATPase per enzyme site (molecular specific activity) in SD cortex was only 29-50% that of controls. In the single sample of SD kidney available, ATPase activity was normal. These data suggest that an attenuation in the brain specific form of NA, K ATPase may be related to the pathogenesis of intralamellar myelin edema in SD. (Supported in part by USPHS grants HD 05515 and HD 04147)
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Lott, I., Reiss, D. & Sapirstein, V. 1149 ALTERED Na, K ATPase IN SPONGY DEGENERATION (CANAVAN'S DISEASE). Pediatr Res 15 (Suppl 4), 634 (1981). https://doi.org/10.1203/00006450-198104001-01175
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DOI: https://doi.org/10.1203/00006450-198104001-01175