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Publication : Prion protein accumulation and neuroprotection in hypoxic brain damage.

First Author  McLennan NF Year  2004
Journal  Am J Pathol Volume  165
Issue  1 Pages  227-35
PubMed ID  15215178 Mgi Jnum  J:91153
Mgi Id  MGI:3046028 Doi  10.1016/S0002-9440(10)63291-9
Citation  McLennan NF, et al. (2004) Prion protein accumulation and neuroprotection in hypoxic brain damage. Am J Pathol 165(1):227-35
abstractText  The function of the normal conformational isoform of prion protein, PrP(C), remains unclear although lines of research have suggested a role in the cellular response to oxidative stress. Here we investigate the expression of PrP(C) in hypoxic brain tissues to examine whether PrP(C) is in part regulated by neuronal stress. Cases of adult cerebral ischemia and perinatal hypoxic-ischemic injury in humans were compared with control tissues. PrP(C) immunoreactivity accumulates within neuronal processes in the penumbra of hypoxic damage in adult brain, and within neuronal soma in cases of perinatal hypoxic-ischemic injury, and in situ hybridization analysis suggests an up-regulation of PrP mRNA during hypoxia. Rodents also showed an accumulation of PrP(C) in neuronal soma within the penumbra of ischemic lesions. Furthermore, the infarct size in PrP-null mice was significantly greater than in the wild type, supporting the proposed role for PrP(C) in the neuroprotective adaptive cellular response to hypoxic injury.
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