ARTICLES
Glucocorticoids, the adrenal steroid hormones secreted during stress,
are essential to neuronal function
1
; however, their excess after brain
injury is detrimental to neuronal survival
2
. These effects are most pro-
nounced in neurons of the hippocampus, a brain region important for
learning and memory and enriched with glucocorticoid receptors.
Glucocorticoids compromise the ability of hippocampal neurons to
survive neurological damage by exacerbating various steps in the excito-
toxic glutamate–calcium–reactive oxygen species cascade
3
. This ability
of glucocorticoids to compromise neuronal survival is of particular
clinical interest because necrotic neurological injuries, such as stroke,
seizure or hypoglycemia, provoke considerable secretion of glucocorti-
coids. In contrast with the action of glucocorticoids, estrogen is neuro-
protective in the aged or injured brain
4
.
On the basis of these observations, we designed three genetic
strategies to counter this hormonal enhancement of injury and
investigate the mechanisms involved: two genes were selected and
engineered to directly modulate or interfere with the regulatory
pathway of glucocorticoids, and a third gene was designed to con-
vert the deleterious effects of glucocorticoid into beneficial estro-
genic ones. Our first two approaches blocked the hormonal signal
by suppressing glucocorticoid-induced translocation of the gluco-
corticoid receptor (GR) to minimize the glucocorticoid-induced
regulation of gene expression and to prevent the consequent exac-
erbation of excitotoxicity. Our third approach both blocked
glucocorticoid-induced GR translocation and converted the glu-
cocorticoid signal into a genomic estrogenic effect. This combina-
tion provided the most neuroprotection, which is mediated
partially through upregulation of brain-induced neurotrophic
factor (BDNF).
RESULTS
Generation and characterization of therapeutic vectors
To identify actions of glucocorticoids that may be useful targets for
therapeutic intervention, we evaluated three different modes of
manipulating the endocrine modulation of neuron death. The first
intervention involved neuronal overexpression of 11β-hydroxysteroid
dehydrogenase (11βHSD) type 2 (Fig. 1a). This renal cytosolic
enzyme unidirectionally hydrolyzes active corticosterone (the pre-
dominant rodent glucocorticoid) to its inactive metabolite corti-
sone
5
, thereby reducing glucocorticoid signaling.
The second intervention was more selective. Although high-stress
levels of glucocorticoids are disruptive, moderate increases in gluco-
corticoid concentrations facilitate synaptic plasticity in the hip-
pocampus and hippocampus-dependent cognition. These opposing
effects of glucocorticoids are mediated by two different receptors
6
.
The high-affinity mineralocorticoid receptor (MR) is nearly saturated
at basal glucocorticoid concentrations, and mediates the beneficial
effects of moderate glucocorticoid elevation
7
. In contrast, the lower-
affinity GR is heavily occupied only at stress-level concentrations of
glucocorticoids and mediates the deleterious effects of glucocorti-
coids. Thus, it is desirable to target GR- but not MR-mediated gluco-
corticoid actions. To do so, we constructed a rat dominant-negative
GR (dnGR; Fig. 1a). No dnGR native to the rat is known; however, an
orthologous dominant-negative splice variant of the human GR has
been characterized, and served as our template. The β isoform of the
human GR
8,9
(GRβ) is identical to the common GR (GRα) for the
first 727 amino acids. However, it lacks the 50 C-terminal residues of
the α isoform, terminating instead with 15 nonhomologous amino
acids. Thus, GRβ cannot bind with ligands or interact strongly with
Departments of
1
Biological Sciences,
2
Neurology and Neurological Sciences,
3
Neurosurgery and
4
Biomedical Informatics, Stanford University, Stanford, California,
USA.
5
Leibniz Institute for Neurobiology, Magdeburg, Germany. Departments of
6
Psychiatry and
7
Infectious Diseases, Stanford University, Stanford, California, USA.
D.K., W.O.O. and Z.S.P. contributed equally to this work. Correspondence should be addressed to D.K. (danielak@stanford.edu).
Published online 8 August 2004; doi:10.1038/nn1296
Restructuring the neuronal stress response with anti-
glucocorticoid gene delivery
D Kaufer
1,3
, W O Ogle
1
, Z S Pincus
1,4
, K L Clark
1
, A C Nicholas
1
, K M Dinkel
1,5
, T C Dumas
1
, D Ferguson
6
,
A L Lee
1
, M A Winters
7
& R M Sapolsky
1,2
Glucocorticoids, the adrenal steroids released during stress, compromise the ability of neurons to survive neurological injury. In
contrast, estrogen protects neurons against such injuries. We designed three genetic interventions to manipulate the actions of
glucocorticoids, which reduced their deleterious effects in both in vitro and in vivo rat models. The most effective of these
interventions created a chimeric receptor combining the ligand-binding domain of the glucocorticoid receptor and the DNA-
binding domain of the estrogen receptor. Expression of this chimeric receptor reduced hippocampal lesion size after
neurological damage by 63% and reversed the outcome of the stress response by rendering glucocorticoids protective rather
than destructive. Our findings elucidate three principal steps in the neuronal stress-response pathway, all of which are
amenable to therapeutic intervention.
NATURE NEUROSCIENCE VOLUME 7 | NUMBER 9 | SEPTEMBER 2004 947
© 2004 Nature Publishing Group http://www.nature.com/natureneuroscience