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Aspartoacylase supports oxidative energy metabolism during myelination.
[canavan disease]
The
inherited
leukodystrophy
Canavan
disease
arises
due
to
a
loss
of
the
ability
to
catabolize
N-
acetylaspartic
acid
(
NAA
)
in
the
brain
and
constitutes
a
major
point
of
focus
for
efforts
to
define
NAA
function
.
Accumulation
of
noncatabolized
NAA
is
diagnostic
for
Canavan
disease
,
but
contrasts
with
the
abnormally
low
NAA
associated
with
compromised
neuronal
integrity
in
a
broad
spectrum
of
other
clinical
conditions
.
Experimental
evidence
for
NAA
function
supports
a
role
in
white
matter
lipid
synthesis
,
but
does
not
explain
how
both
elevated
and
lowered
NAA
can
be
associated
with
pathology
in
the
brain
.
We
have
undertaken
a
systematic
analysis
of
postnatal
development
in
a
mouse
model
of
Canavan
disease
that
delineates
development
and
pathology
by
identifying
markers
of
oxidative
stress
preceding
oligodendrocyte
loss
and
dysmyelination
.
These
data
suggest
a
role
for
NAA
in
the
maintenance
of
metabolic
integrity
in
oligodendrocytes
that
may
be
of
relevance
to
the
strong
association
between
NAA
and
neuronal
viability
.
N-
acetylaspartic
acid
is
proposed
here
to
support
lipid
synthesis
and
energy
metabolism
via
the
provision
of
substrate
for
both
cellular
processes
during
early
postnatal
development
.
