Gene expression and oligodendrocyte development in the myelin deficient rat.
The proteolipid proteins play a major role in the structure of the CNS myelin sheath, but they have also been implicated in the oligodendrocyte development leading to myelination. Mutations in the PLP gene result in severe dysmyelination and a paucity of mature oligodendrocytes. The myelin deficient (md) rat, carrying a Thr75-->Pro substitution present in both isoforms of proteolipid protein (PLP and DM20), is the most severely affected of the PLP mutants described to date. The expression of myelin associated genes was quantitated to determine the effect of the mutation on oligodendrocyte development in vivo. At 5 days postnatal, gene expression in the md rat approximated that in age-matched control rats, but as they matured, there was a progressive inhibition of gene expression in the md rats. The genes expressed late in the myelination program (PLP and MBP) were affected more dramatically than those expressed earlier in oligodendrocyte development (CNP and GPDH). The results indicate that the later stages of oligodendrocyte maturation and myelin elaboration are inhibited.
Nadon
N L
NL
Biology Dept, University of Tulsa, OK 74104, USA.
Duncan
I D
ID
D000818:Animals; D001059:Apoproteins / Q000172:deficiency* / Q000235:genetics; D001253:Astrocytes / Q000737:chemistry / Q000502:physiology; D001483:Base Sequence; D002455:Cell Division / Q000187:drug effects; D015870:Gene Expression / Q000502:physiology; D005904:Glial Fibrillary Acidic Protein / Q000032:analysis; D008297:Male; D008969:Molecular Sequence Data; D009185:Myelin Proteins / Q000172:deficiency* / Q000235:genetics; D018991:Myelin Proteolipid Protein / Q000172:deficiency* / Q000235:genetics; D009186:Myelin Sheath / Q000235:genetics / Q000502:physiology*; D009419:Nerve Tissue Proteins / Q000235:genetics; D050816:Octamer Transcription Factor-6; D009836:Oligodendroglia / Q000166:cytology* / Q000502:physiology; D051381:Rats; D011922:Rats, Mutant Strains; D014157:Transcription Factors / Q000235:genetics
D001059:Apoproteins; D005904:Glial Fibrillary Acidic Protein; D009185:Myelin Proteins; D018991:Myelin Proteolipid Protein; D009419:Nerve Tissue Proteins; C489071:Plp1 protein, rat; C494179:Pou3f1 protein, rat; D014157:Transcription Factors; D050816:Octamer Transcription Factor-6