Activity of 2'3'-cyclic-nucleotide 3'-phosphodiesterase and content of PO protein in the peripheral nervous system of the dystrophic mouse and chicken.
To investigate whether various myelin markers could detect pathological changes in myelination, the activity of 2'-3'-cyclic nucleotide 3'-phosphodiesterase (CNP) and the level of PO protein were compared in the peripheral nervous system (PNS) of the dystrophic mouse and chicken and their phenotypically normal siblings. The same comparison was made for subcellular fractions of these nerves. The level of PO protein and the activity of CNP were normal in the spinal roots of sciatic nerves of the 129B6F1/Jdy/dy strain of dystrophic mouse. These parameters were slightly but significantly lower in the spinal roots of the 129REJ/dy/dy strain of dystrophic mouse. These results suggest that the 129B6F1/Jdy/dy mutant and the 129REJ/dy/dy mutant mouse might not have the same type of biochemical abnormality in their PNS. The specific activity of CNP and the proportion of PO protein increased significantly in the various regions of the PNS of 1-day-old chicks when compared to that of the 18-day-old embryos. Subcellular fractionation of peripheral nerves indicated that these components were enriched in the crude myelin fraction. These results support the conclusion that PO protein and CNP are suitable markers of myelinogenesis in the PNS of the chick. These parameters were normal in the line 413 dystrophic chicken. These results do not support the conclusion of a systemic membrane defect in the PNS of the dystrophic mouse and chicken.
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D063309:2',3'-Cyclic Nucleotide 3'-Phosphodiesterase; D015087:2',3'-Cyclic-Nucleotide Phosphodiesterases / Q000378:metabolism*; D000818:Animals; D002645:Chickens; D051379:Mice; D008817:Mice, Mutant Strains; D009136:Muscular Dystrophies / Q000235:genetics / Q000378:metabolism* / Q000503:physiopathology; D018993:Myelin P0 Protein; D009185:Myelin Proteins / Q000378:metabolism*; D009186:Myelin Sheath / Q000502:physiology; D010525:Peripheral Nerves / Q000503:physiopathology; D010727:Phosphoric Diester Hydrolases; D013347:Subcellular Fractions / Q000378:metabolism
D018993:Myelin P0 Protein; D009185:Myelin Proteins; D015087:2',3'-Cyclic-Nucleotide Phosphodiesterases; D010727:Phosphoric Diester Hydrolases; D063309:2',3'-Cyclic Nucleotide 3'-Phosphodiesterase; C571338:Cnp protein, mouse