2'-3'-cyclic nucleotide 3'-phosphohydrolase activity in the central nervous system of a myelin deficient mutant (Shiverer)

Abstract

Shiverer is a recessive autosomal mutant characterized by the deficiency of the myelin in the central nervous system. 2',3'-Cyclic nucleotide 3'-phosphohydrolase (CNPase) activity of the various parts of the central nervous system from the Shiverer did not differ significantly from those of the control. The analysis of the protein profiles of the purified myelin from the Shiverer showed a greatly decreased proportion of proteolipid protein, and almost complete absence of small and large basic proteins, and intermediate protein. Woldgram protein accounted for a much larger percentage of the total myelin protein than is the case in myelin from the control. Proteolipid protein, small and large basic proteins, and intermediate protein were found to be undetectable or decreased in various parts of the central nervous system from the Shiverer. Morphological observation by optic and electron microscope showed that the myelination of the optic nerve was equally affected as the spinal cord.