prions

[Graeme Price]

In article <316D0474.2B91 at pen.gulbenkian.pt>, jcosta at pen.gulbenkian.pt wrote:

> Tim Fitzmaurice wrote:
> > 
> > On 10 Apr 1996, Ian A. York wrote:
> > > I've assumed that the spread to the brain is within cells of the
> > > reticuloenbdothelial system - via monocytes/macrophages.  I suppose it
> > Would travel in this kind of cell not make it far more transmissible than
> > this kind of agent appears to be?
> > 
> > Has anyon seen any mention in any paper of a putative normal function of PrP
> > gene products at all?
> > 
> > Tim
> 
> A Portuguese newspaper reports today that a paper from a Japanese group
> (S. Katamine, from U. Nagasaki) is going to appear today (where?) des-
> cribing a deffect in cerebelous functions in transgenic mice with
> knocked out PRP genes. Late in life, these mice show atrophy of the
> cerebelum, with massive death of Purkinje cells.
> These resuts are contradictory to the ones from Prusiner's group, that
> were unable to detect any defficiency in knocked out mice.

The reference is in this week's Nature:

Sakaguchi et al. (1996) Loss of cerebellar Purkinje cells in aged mice
homozygous for a disrupted PrP gene. Nature 380(11 April): 528-531 

The mice developed a progressive ataxia at approx 70 weeks (although
appeared normal till then), with loss of Purkinje cells, but other PrP-
mouse strains did not (although the authors are unsure why). The
implication that is drawn from all this is that PrP may have a role in
Purkinje cell survival (in the long term). 

Hope this helps a little

Graeme

-- 
Graeme Price
Microbial Molecular Genetics and Cell Biology Group, 
School of Biological Sciences, Biology West Building,
University of Birmingham,
Edgbaston, Birmingham,
West Midlands, B15 2TT.
United Kingdom.

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