2000年11月
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
AMERICAN JOURNAL OF PATHOLOGY
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- 巻
- 157
- 号
- 5
- 開始ページ
- 1447
- 終了ページ
- 1452
- 記述言語
- 英語
- 掲載種別
- 出版者・発行元
- AMER SOC INVESTIGATIVE PATHOLOGY, INC
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.
- リンク情報
- ID情報
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- ISSN : 0002-9440
- Web of Science ID : WOS:000165226600004