NR AHFW
AU Li,A.; Sakaguchi,S.; Shigematsu,K.; Atarashi,R.; Roy,B.C.; Nakaoke,R.; Arima,K.; Okimura,N.; Kopacek,J.; Katamine,S.
TI Physiological expression of the gene for PrP-like protein, PrPLP/Dpl, by brain endothelial cells and its ectopic expression in neurons of PrP-deficient mice ataxic due to Purkinje cell degeneration
QU American Journal of Pathology 2000 Nov; 157(5): 1447-52
PT journal article
AB Recently, a novel gene encoding a prion protein (PrP)-like glycoprotein, PrPLP/Dpl, was identified as being expressed ectopically by neurons of the ataxic PrP-deficient (PRNP(-/-)) mouse lines exhibiting Purkinje cell degeneration. In adult wild-type mice, PrPLP/Dpl mRNA was physiologically expressed at a high level by testis and heart, but was barely detectable in brain. However, transient expression of PrPLP/Dpl mRNA was detectable by Northern blotting in the brain of neonatal wild-type mice, showing maximal expression around 1 week after birth. In situ hybridization paired with immunohistochemistry using anti-factor VIII serum identified brain endothelial cells as expressing the transcripts. Moreover, in the neonatal wild-type mice, the PrPLP/Dpl mRNA colocalized with factor VIII immunoreactivities in spleen and was detectable on capillaries in lamina propria mucosa of gut. These findings suggested a role of PrPLP/Dpl in angiogenesis, in particular blood-brain barrier maturation in the central nervous system. Even in the ataxic Ngsk PRNP(-/-) mice, the physiological regulation of PrPLP/Dpl mRNA expression in brain endothelial cells was still preserved. This strongly supports the argument that the ectopic expression of PrPLP/Dpl in neurons, but not deregulation of its physiological expression in endothelial cells, is involved in the neuronal degeneration in ataxic PRNP(-/-) mice.
MH Adenosinetriphosphatase; Animal; Ataxia/etiology/*genetics; Cerebrovascular Circulation/physiology; Endothelium, Vascular/cytology/physiology; Fungal Proteins; *Gene Expression; Mice; Mice, Mutant Strains; Nerve Degeneration/complications; Neurons/physiology; Prions/*genetics/*metabolism; Purkinje Cells/physiology; Support, Non-U.S. Gov't
AD Department of Molecular Microbiology and Immunology, Nagasaki University Graduate School of Medical Sciences, Nagasaki, Japan.
SP englisch
PO USA
EA pdf-Datei und HTML-Version