In Brief
Australian decision on stem
cell use
Australian Prime Minister John Howard,
has produced an agreement between
himself and the Australian state and
territory leaders on the use of stem cells and
cloning in humans. Scientists will have the
right to use spare embryos from in vitro
fertilization programmes for research, but
nuclear transfer technology and therapeutic
cloning will be banned. An ethics committee
will consider how to ensure that embryos
are created only for in vitro fertilization
and not just for research. The Australian
5April (2002), p. 4 DM
Yeast ‘localizome’ in vitro
Anuj Kumar et al., report on a large scale
analysis of proteins (the proteome) in
baker’s yeast, Saccharomyces cerevisiae.
Although the S. cerevisiae genome
sequence was completed in 1996, over a
third of the protein products encoded by
the 6000 genes are yet to be assigned a
function. Because subcellular localization is
a strong indicator of protein function, a
high-throughput method to tag individual
proteins and visualize their movements
within living cells was developed,
successfully determining the subcellular
location – ‘localizome’ – of over 2700 yeast
proteins.The work provided an insight into
the potential function of nearly half of all
previously uncharacterized yeast proteins.
For more information there is a database
containing the results of the work:
http://ygac.med.yale.edu. (Genes Dev. 16,
707–719) DM
Sleeping beauty transformed
For the first time, a transposon (called
Sleeping Beauty) has been used to
genetically modify a vertebrate animal.
Adam J. Dupuy et al. injected the
transposon containing the gene for a yellow
coat colour into a mouse embryo, resulting
in a genetically modified mouse. ‘We’re very
excited about Sleeping Beauty’s potential,’
said David Largaespada, group leader for
the project. ‘One use would be to add genes
to germ cells or early embryos in order to
produce large amounts of a protein in an
animal.The protein then would be purified
and used as a drug treatment for
hemophilia, for instance.’ Largaespada is
TRENDS in Biotechnology Vol.20 No.6 June 2002
http://tibtech.trends.com 0167-7799/02/$ – see front matter © 2002 Elsevier Science Ltd. All rights reserved.
235 News& Comment
Cellular prion protein (PrP
C
) is a cell-surface
protein expressed mostly by neural tissue.
Although its precise function is unknown,
among the possibilities is a role in copper
metabolism. An abnormal conformation of
the protein (PrP
Sc
) is believed to be the
causative agent of prion diseases.These are
fatal neurodegenerative disorders in which
neuronal loss and overgrowth of glial fibres
occur after long, seemingly asymptomatic,
incubation periods. It seems that expression
of PrP
C
is necessary for the manifestation of
prion disease, as genetically modified mice
that fail to express it are resistant to
infection with mouse prion inoculum.
However, exactly how the conformational
change from PrP
C
to PrP
Sc
leads to
neurodegeneration is not understood.
PrP
C
is a copper-binding protein.To
further elucidate the role of metals in prion
disease,Thackray et al. [1] investigated
whether there were changes in trace
elements in the brains, and other organs,
of mice infected with mouse scrapie.
Interestingly, the authors found changes in
the levels of copper and manganese in the
brains of infected mice, prior to the onset of
clinical symptoms. Specifically, they
observed a reduction in brain copper and
significantly elevated levels in the liver and
blood, suggesting a displacement of copper
from the brain or other tissues. Changes in
manganese were more widespread, with
elevated levels occurring in blood, brain
and muscle. Of particular note was the
increase in brain manganese relative to the
decrease in copper.The change reached its
maximum at the onset of clinical signs.This
constitutes the first report of a
neurodegenerative disease in which there is
a systemic change in manganese, perhaps
signifying a characteristic that will allow
selective diagnosis of prion disease.
It has been suggested that prions are
antioxidant proteins.Therefore, the authors
investigated whether there were also
changes in antioxidant proteins associated
with disease progression.They found that
whereas expression levels of both
copper/zinc-superoxide dismutase (SOD)
and manganese-SOD are not altered by
prion disease, the activity of the enzymes is
affected. Both purified and recombinant
PrP
C
are believed to have a catalytic activity
similar to SOD, which is a copper-dependent
enzyme.The metal content and level of
PrP-dependent SOD activity in purified
proteins was therefore assessed. During
infection, inoculated mice showed a loss of
bound copper and an increase in associated
manganese, together with a significant
decrease in SOD-like activity.The decrease
in activity matched the decrease in copper,
implying a reduction in copper and
antioxidant activity of the total PrP content.
Manganese binding to PrP
C
is known to
stimulate its conversion into an abnormal
conformation rich in β-sheets, and it was
thought that an imbalance in brain trace
elements could lead to the formation of PrP
Sc
.
This study suggests that scrapie-induced
prion disease might cause such a change in
the brain, and therefore a metal imbalance
could induce conversion of PrP
C
into PrP
Sc
.
Thackray et al. therefore postulate that
alterations in trace-element metabolism
arising from changes in metal binding to PrP
are central to the pathological modifications
characteristic of prion disease. Further
analysis of the alterations are necessary,
but the implication is that these values
might represent specific changes with
immediate diagnostic potential.
1 Thackray, A.M. et al. (2002) Metal imbalance and
compromised antioxidant function are early
changes in prion disease. Biochem. J.
362, 253–258
Teresa K.Attwood
attwood@bioinf.man.ac.uk
M etals in prion disease