Factors associated with predisposition and vulnera-
bility to neurodegenerative disorders may be
described usefully within the context of gene-envi-
ronment interplay. There are many identified genet-
ic determinants for so-called genetic disorders, and
it is possible to duplicate many elements of recog-
nized human neurodegenerative disorders in either
knock-in or knock-out mice. However, there are
similarly, many identifiable environmental influ-
ences on outcomes of the genetic defects; and the
course of a progressive neurodegenerative disorder
can be greatly modified by environmental elements.
Constituent cellular defense mechanisms responsive
to the challenge of increased reactive oxygen species
represent only one crossroad whereby environment
can influence genetic predisposition. In this paper
we highlight some of the major neurodegenerative
disorders and discuss possible links of gene-environ-
ment interplay. The process of adult neurogenesis in
brain is also presented as an additional element that
influences gene-environment interplay. And the so-
called priming processes (i.e., production of recep-
tor supersensitization by repeated drug dosing), is
introduced as yet another process that influences
how genes and environment ultimately and co-
dependently govern behavioral ontogeny and out-
come. In studies attributing the influence of genetic
alteration on behavioral phenotypy, it is essential to
carefully control environmental influences.
Keywords: Neurodevelopment Neurodegeneration;
Neurodevelopment; Neurogenesis; Alzheimer disease;
Huntington disease; Tau neuropathy; Priming
INTRODUCTION
Most of the neurodegenerative disorders that have
received some degree of documentation, such as
Alzheimer's disease (AD), Parkinson's disease (PD),
amyotrophic lateral sclerosis (ALS) and Huntington's
disease (HD) are characterized by neuronal damaged
caused putatively by toxic, abnormal, aggregate-prone
proteins or 'clusters'. A recent review by Petrucelli and
Dawson (2004) outlines and describes three different
aspects of neurodegenerative mechanisms involved in
these disorders, as follows: (1) the genetic configura-
tion underlying the abnormal processing and accumu-
lation of misfolded proteins in the neurodegenerative
diseases, using PD as a model disorder, (2) an under-
standing and consideration of the cellular mechanisms
for disposal of abnormal proteins, and the effects of
toxic protein accumulation on the ubiquitin proteasome
system and neuronal survival, and (3) the development
and challenges offered by cell culture and animal mod-
els leading to rational and effective treatment strate-
gies. In the realm of neurodegeneration, there are
known genetically-associated disorders such as
ALS/motor neuron disease, HD, early-onset PD, myas-
thenia gravis, and others. ALS/motor neuron disease
can be inherited or acquired by consumption of foods
containing a high content of abnormal excitatory amino
acids. HD is an autosomal dominant disorder, caused
by abnormal expansion in the length of a CAG triplet
repeat sequence in a gene on chromosome 4 (i.e., the
huntingtin gene). Myasthenia gravis can be autosomal
and dominantly inherited; or caused by thymus-derived
F.P. Graham Publishing Co.
Neurotoxicity Research, 2004, VOL. 6(6). pp. 415-434
Gene-Environment Interplay in Neurogenesis
and Neurodegeneration
TOMÁS PALOMO
a
, TREVOR ARCHER
b
, RICHARD J. BENINGER
c
and RICHARD M. KOSTRZEWA
d,
*
a
Servicio Psiquiátrico, Hospital Universitario 12 de Octubre, Avda. de Córdoba s/n, 28041 Madrid, Spain;
b
Department of
Psychology, University of Göteborg, Box 500, SE-40530 Göteborg, and Department of Health and Behavioural Science,
University of Kalmar, Kalmar, Sweden;
c
Departments of Psychology and Psychiatry, Queen's University, Kingston ON K7L
3N6, Canada;
d
Department of Pharmacology, Quillen College of Medicine, East Tennessee State University, Johnson City,
TN 37614, USA. kostrzew@etsu.edu
(Received 30 September 2004; Revised 19 October 2004; In final form 23 November 2004)
*Corresponding author. Tel.: 1 423 439-6321; Fax: 1 423 439-8773; E-mail: Kostrzew@etsu.edu
ISSN 1029 8428 print/ ISSN 1476-3524 online. © 2004 FP Graham Publishing Co., www.NeurotoxicityResearch.com