Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: EC:3.1.4.37 (CNPase)
 539 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Patients with Pelizaeus-Merzbacher disease (PM), hemizygous mice with the jimpy mutation (jp/Y), and hemizygous rats with X-linked myelin deficiency (md/Y) share a profound lack of proteolipid protein (PLP) in their central nervous systems (CNS). The peripheral nervous system is normal. These X-linked disorders are associated with or actually caused by the lack of normal oligodendrocytes. Vibratome sections of brain were incubated with antisera to myelin basic protein (MBP), myelin-associated glycoprotein (MAG), 2':3'-cyclic-nucleotide 3'-phosphodiesterase (CNP) (EC 3.1.4.37), PLP, a synthetic PLP-peptide, glial fibrillary acidic protein (GFAP), and transferrin. Reaction product was developed by sequential incubation with biotinylated second antibodies, the avidin-biotin-peroxidase complex (ABC), and diaminobenzidine (DAB) plus hydrogen peroxide as chromogenic substrates. In PM, jp/Y and md/Y, islands of myelin-like structures were revealed by antisera to MBP, MAG, and CNP. Reaction product after application of anti-PLP was absent. Reaction product after anti-PLP-peptide was restricted to infrequent bizarre cells possibly representing abnormal oligodendroglia. The lack of oligodendrocytes in jp/Y and md/Y could also be confirmed by immunocytochemistry for transferrin.
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PMID:Comparative immunocytochemistry of Pelizaeus-Merzbacher disease, the jimpy mouse, and the myelin-deficient rat. 245 99

Peripheral nerves of the shiverer mouse, which are characterized by the absence of major dense lines and myelin basic proteins in CNS myelin, were analyzed. From subcellular fractionation of sciatic nerves, it was found from the SDS-polyacrylamide gel electrophoresis that the Pl and Pr proteins equivalent to myelin basic protein of CNS and PM protein were missing in the shiverer in both P2A and P3A fractions in which PNS myelin is recovered. No extra bands were observed in any other fractions of the shiverer in place of the absence of the proteins. The activities of 2',3'-cyclic nucleotide 3'-phosphohydrolase (CNPase) of P2A and P3A fractions were high, but that of the P3X fraction which floated over 0.32 M sucrose was the highest among the fractions examined in both the shiverer and the control. Developmental analysis of the protein profiles revealed that PO, Pl, Pr and PM proteins increased rapidly from the sixth day postnatally up to the twentieth day after birth in the control. No differences were observed between the shiverer and the control as for PO protein, but Pl, Pr and PM proteins were absent in the shiverer throughout the development. The CNPase activity of total homogenate of sciatic nerve fibers at birth in the control showed high activity comparable to that of the adult value, but there was no significant difference in activity between the control and the shiverer at any stage of development. Immunohistochemical reaction using peroxidase anti-peroxidase method showed that the myelin from the shiverer did not react with the MBP antiserum, while that of the control reacted positively. On the contrary, the myelin from both shiverer and the control reacted positively against P2 antibody.
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PMID:Peripheral nervous system of shiverer mutant mice: developmental change of myelin components and immunohistochemical demonstration of the absence of MBP and presence of P2 protein. 618 16

We describe the immunohistochemical localization for brain 2', 3'-cyclic nucleotide 3'-phospho diesterase (EC 3.1.4.37) by the peroxidase-antiperoxidase method. Myelinated fibre tracts were immunohistochemically stained in 15-day-old rat cerebrum and spinal cord; at high magnification, myelinated nerve fibres and immature glial cells with their processes were shown to be stained.
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PMID:Immunohistochemical localization of 2', 3'-cyclic nucleotide 3'-phosphodiesterase in the central nervous system. 626 88