Expression of myelin-specific proteins during development of normal and hypomyelinated Paralytic tremor mutant rabbits. I. Studies on the brain homogenates.
The paralytic tremor (pt) rabbit is an X-linked recessive mutant characterized by hypomyelination of the CNS. The onset of myelin mutants' neurological symptoms typically occurs about the tenth postnatal day. A partial recovery is often observed; thus, the life-span of affected animals is almost normal and they can breed successfully. Mutants presenting this phenotype were chosen for our study. Because proteins can serve as excellent markers for the myelin formation process, we examined the developmental expression of several important myelin proteins (PLP/DM-20, MBP, CNP, MAG, and MOG) in both pt mutant and control rabbit brain homogenates. Expression of the investigated proteins occurs in rabbits as follows: CNP and MAG are already present at the early postnatal stage; PLP/DM-20 and MBP appear about the 10th postnatal day; MOG, expressed last, has been detected on the 28th postnatal day. Whereas the MBP, CNP, MAG, and MOG content is only slightly reduced in mature pt mutant brain homogenates (80-90% of control values), the amount of PLP corresponds to approximately 30-40% of that present in controls. Expression of all of the examined proteins is substantially retarded in maturing brains, which leads to the conclusion that besides severe PLP deficiency, retardation of oligodendrocyte maturation is another probable feature of pt mutation.
Sypecka
J
J
Department of Neurochemistry, Polish Academy of Sciences, Warsaw, Poland.
Domańska-Janik
K
K
D000818:Animals; D001921:Brain / Q000254:growth & development / Q000378:metabolism*; D009154:Mutation; D009185:Myelin Proteins / Q000096:biosynthesis*; D009186:Myelin Sheath / Q000502:physiology*; D010243:Paralysis / Q000235:genetics / Q000378:metabolism*; D011817:Rabbits; D012016:Reference Values; D014202:Tremor / Q000235:genetics / Q000378:metabolism*
D009185:Myelin Proteins