MESSENGER-RNA LEVELS FOR CENTRAL-NERVOUS-SYSTEM MYELIN PROTEINS IN MYELIN DEFICIENCY OF CAPRINE BETA-MANNOSIDOSIS

Caprine beta-mannosidosis is an inherited lysosomal storage disease th at leads to a deficiency of oligodendrocytes and hypomyelination. Our previous results demonstrated that low levels of myelin-associated gly coprotein (MAG), 2',3'-cyclic nucleotide 3'-phosphodiesterase (CNP) an d proteolipid protein (PLP) found in CNS samples correlated with decre ased yields of myelin. However, there was a relative preservation of m yelin basic protein (MBP) in the spinal cord samples of affected goats . This report shows that the amounts of myelin protein mRNAs in the sp inal cords of affected goats relative to control goats are also decrea sed. The levels of mRNA for MAG, MBP and PLP in affected goat spinal c ords compared with those of controls were equally decreased to approxi mately 50% for the three myelin proteins. This suggests that the relat ive preservation of MBP protein in the spinal cords is not due to a hi gher MBP mRNA level, but might be due to a difference in post-transcri ptional processes.