Rare Diseases Symptoms Automatic Extraction
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Dual transgenic reporter mice as a tool for monitoring expression of glial fibrillary acidic protein.
[alexander disease]
Glial
fibrillary
acidic
protein
(
GFAP
)
is
the
major
intermediate
filament
protein
of
astrocytes
,
and
its
expression
changes
dramatically
during
development
and
following
injury
.
To
facilitate
study
of
the
regulation
of
GFAP
expression
,
we
have
generated
dual
transgenic
mice
expressing
both
firefly
luciferase
under
the
control
of
a
2
.
2
kb
human
GFAP
promoter
and
Renilla
luciferase
under
the
control
of
a
0
.
5
kb
human
Glyceraldehyde
3
phosphate
dehydrogenase
(
GAPDH
)
promoter
for
normalization
of
the
GFAP
signal
.
The
GFAP
-fLuc
was
highly
expressed
in
brain
compared
to
other
tissues
,
and
was
limited
to
astrocytes
,
whereas
the
GAPDH
-RLuc
was
more
widely
expressed
.
Normalization
of
the
GFAP
signal
to
the
GAPDH
signal
reduced
the
inter-individual
variability
compared
to
using
the
GFAP
signal
alone
.
The
GFAP
/
GAPDH
ratio
correctly
reflected
the
up-regulation
of
GFAP
that
occurs
following
retinal
degeneration
in
FVB
/
N
mice
because
of
the
rd
mutation
.
Following
kainic
acid-induced
seizures
,
changes
in
the
GFAP
/
GAPDH
ratio
precede
those
in
total
GFAP
protein
.
In
knock-
in
mice
expressing
the
R
236
H
Alexander
disease
mutant
,
GFAP
promoter
activity
is
only
transiently
elevated
and
may
not
entirely
account
for
the
accumulation
of
GFAP
protein
that
takes
place
.
