title: mRNA levels for central nervous system myelin proteins in myelin deficiency of caprine beta-mannosidosis.
abstract: Caprine beta-mannosidosis is an inherited lysosomal storage disease that leads to a deficiency of oligodendrocytes and hypomyelination. Our previous results demonstrated that low levels of myelin-associated glycoprotein (MAG), 2',3'-cyclic nucleotide 3'-phosphodiesterase (CNP) and proteolipid protein (PLP) found in CNS samples correlated with decreased yields of myelin. However, there was a relative preservation of myelin basic protein (MBP) in the spinal cord samples of affected goats. This report shows that the amounts of myelin protein mRNAs in the spinal cords of affected goats relative to control goats are also decreased. The levels of mRNA for MAG, MBP and PLP in affected goat spinal cords compared with those of controls were equally decreased to approximately 50% for the three myelin proteins. This suggests that the relative preservation of MBP protein in the spinal cords is not due to a higher MBP mRNA level, but might be due to a difference in post-transcriptional processes.
authors.affiliation: Demyelinating Disorders Unit, NINDS, NIH, Bethesda, MD 20892, USA.
authors.forename: M
authors.initials: M
authors.lastname: Sasaki
authors.forename: K L
authors.initials: KL
authors.lastname: Lovell
authors.forename: J R
authors.initials: JR
authors.lastname: Möller
mesh_terms: D015087:2',3'-Cyclic-Nucleotide Phosphodiesterases / Q000172:deficiency; D000818:Animals; D015152:Blotting, Northern; D002490:Central Nervous System / Q000378:metabolism*; D003711:Demyelinating Diseases / Q000235:genetics / Q000378:metabolism / Q000662:veterinary*; D015511:Goat Diseases / Q000378:metabolism*; D006041:Goats; D004676:Myelin Basic Protein / Q000096:biosynthesis / Q000172:deficiency; D009185:Myelin Proteins / Q000096:biosynthesis* / Q000172:deficiency / Q000235:genetics*; D018991:Myelin Proteolipid Protein / Q000096:biosynthesis / Q000172:deficiency; D018992:Myelin-Associated Glycoprotein / Q000096:biosynthesis / Q000172:deficiency; D012333:RNA, Messenger / Q000378:metabolism*; D013116:Spinal Cord / Q000378:metabolism / Q000473:pathology; D008363:alpha-Mannosidosis / Q000235:genetics / Q000378:metabolism / Q000662:veterinary*
chemical_list: D004676:Myelin Basic Protein; D009185:Myelin Proteins; D018991:Myelin Proteolipid Protein; D018992:Myelin-Associated Glycoprotein; D012333:RNA, Messenger; D015087:2',3'-Cyclic-Nucleotide Phosphodiesterases