Myelin composition and activities of CNPase and Na+,K+-ATPase in hypomyelinated "pt" mutant rabbit.
A disorder of CNS myelination was found in paralytic tremor ("pt") rabbits. The condition is inherited in a sex-linked recessive mode. Ultrastructurally, an obvious myelin deficiency with aberration of myelin sheath formation is observed. The yield of myelin isolation was reduced to 20-30% of control. Myelin isolated from 4-week-old "pt" rabbits contained reduced amounts of galactosphingolipids and of several myelin protein markers. Moreover, myelin basic protein, analyzed by two-dimensional gel electrophoresis, showed a deficit in its more basic components. All these facts suggest a delay in myelin maturation. Ganglioside content was increased as well as Na+,K+-ATPase specific activity. 2',3'-Cyclic nucleotide phosphodiesterase (CNPase) specific activity was the same in "pt" as in control myelin but differed by having greater sensitivity to detergent activation.
Domańska-Janik
K
K
Department of Neurochemistry, Polish Academy of Sciences, Warsaw, Poland.
Gajkowska
B
B
de Néchaud
B
B
Bourre
J M
JM
D015087:2',3'-Cyclic-Nucleotide Phosphodiesterases / Q000378:metabolism*; D000818:Animals; D002554:Cerebrosides / Q000032:analysis; D003711:Demyelinating Diseases / Q000235:genetics / Q000378:metabolism* / Q000473:pathology; D005227:Fatty Acids / Q000032:analysis; D038983:Galactolipids; D005732:Gangliosides / Q000032:analysis; D006017:Glycolipids / Q000032:analysis; D006900:Hydroxylation; D008854:Microscopy, Electron; D009185:Myelin Proteins / Q000032:analysis; D009186:Myelin Sheath / Q000032:analysis / Q000378:metabolism* / Q000473:pathology; D009900:Optic Nerve / Q000473:pathology; D011817:Rabbits; D000254:Sodium-Potassium-Exchanging ATPase / Q000378:metabolism*
D002554:Cerebrosides; D005227:Fatty Acids; D038983:Galactolipids; D005732:Gangliosides; D006017:Glycolipids; D009185:Myelin Proteins; D015087:2',3'-Cyclic-Nucleotide Phosphodiesterases; D000254:Sodium-Potassium-Exchanging ATPase