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. 2000 Nov;157(5):1447-52.
doi: 10.1016/S0002-9440(10)64782-7.

"VSports" 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

A Li  1 , S Sakaguchi, K Shigematsu, R Atarashi, B C Roy, R Nakaoke, K Arima, N Okimura, J Kopacek, S Katamine
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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

V体育官网 - A Li et al. Am J Pathol. 2000 Nov.

Abstract

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 VSports手机版. .

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Figures

Figure 1.
Figure 1.
Northern blotting for PrPLP/Dpl and PrP mRNAs in various tissues of 9-week-old wild-type (Ngsk Prnp+/+, A) and NgsK Prnp−/− mice (B) using the indicated probes. s. muscle, skeletal muscle.
Figure 2.
Figure 2.
Northern blotting for PrPLP/Dpl and PrP mRNAs in the brain of wild-type (Ngsk Prnp+/+) mice (A) and of NgsK Prnp−/− mice (D) at ages of 1, 3, 6, and 12 days and 8 weeks after birth. PrPLP/Dpl mRNA was transiently expressed in the former soon after birth, but the expression was deregulated in the latter due to intergenic splicing. In situ hybridization for PrPLP/Dpl mRNA with (C and F) or without (B and E) co-immunostaining of factor VIII, a marker of endothelial cells, on the brain sections of Ngsk Prnp+/+ (B and C) and Prnp−/− (E and F) mice. The blue and red stains represent PrPLP/Dpl mRNA and factor VIII immunoreactivity, respectively. Linear or patchy hybridization signals colocalized with factor VIII immunoreactivities are observed throughout the brain of 6-day-old Ngsk Prnp+/+ mouse (B and C), whereas the 6-day-old Ngsk Prnp−/− mouse brain shows the PrPLP/Dpl mRNA expression in neurons in addition to endothelial cells (arrows in E). In the brain of 8-week-old Ngsk Prnp−/− mice (F), there is no PrPLP/Dpl mRNA signal colocalized with the factor VIII immunoreactivity despite its ectopic expression in neurons. Py, pyramidal cells in the hippocampus. Original magnifications, ×100 (B, C, and E) and ×80 (F).
Figure 3.
Figure 3.
In situ hybridization for PrPLP/Dpl mRNA (A) and immunohistochemical staining with rabbit antiserum against factor VIII-related peptide (B) on consecutive sections of the spleen of 6-day-old neonatal wild-type mice. Arrowheads indicate the same sites on each section. WP, white pulp. C: Negative control of in situ hybridization using the sense cRNA probe of PrPLP/Dpl. D: In situ hybridization for PrPLP/Dpl mRNA on a section of gut from 6-day-old neonatal wild-type mice. Arrow indicates Peyer’s node (PN). Original magnifications, ×66 (A−C) and ×55 (D).
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References (VSports app下载)

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