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N-Acetylaspartate in the CNS: from neurodiagnostics to neurobiology.
[canavan disease]
The
brain
is
unique
among
organs
in
many
respects
,
including
its
mechanisms
of
lipid
synthesis
and
energy
production
.
The
nervous
system-
specific
metabolite
N-
acetylaspartate
(
NAA
)
,
which
is
synthesized
from
aspartate
and
acetyl-coenzyme
A
in
neurons
,
appears
to
be
a
key
link
in
these
distinct
biochemical
features
of
CNS
metabolism
.
During
early
postnatal
central
nervous
system
(
CNS
)
development
,
the
expression
of
lipogenic
enzymes
in
oligodendrocytes
,
including
the
NAA-degrading
enzyme
aspartoacylase
(
ASPA
)
,
is
increased
along
with
increased
NAA
production
in
neurons
.
NAA
is
transported
from
neurons
to
the
cytoplasm
of
oligodendrocytes
,
where
ASPA
cleaves
the
acetate
moiety
for
use
in
fatty
acid
and
steroid
synthesis
.
The
fatty
acids
and
steroids
produced
then
go
on
to
be
used
as
building
blocks
for
myelin
lipid
synthesis
.
Mutations
in
the
gene
for
ASPA
result
in
the
fatal
leukodystrophy
Canavan
disease
,
for
which
there
is
currently
no
effective
treatment
.
Once
postnatal
myelination
is
completed
,
NAA
may
continue
to
be
involved
in
myelin
lipid
turnover
in
adults
,
but
it
also
appears
to
adopt
other
roles
,
including
a
bioenergetic
role
in
neuronal
mitochondria
.
NAA
and
ATP
metabolism
appear
to
be
linked
indirectly
,
whereby
acetylation
of
aspartate
may
facilitate
its
removal
from
neuronal
mitochondria
,
thus
favoring
conversion
of
glutamate
to
alpha
ketoglutarate
which
can
enter
the
tricarboxylic
acid
cycle
for
energy
production
.
In
its
role
as
a
mechanism
for
enhancing
mitochondrial
energy
production
from
glutamate
,
NAA
is
in
a
key
position
to
act
as
a
magnetic
resonance
spectroscopy
marker
for
neuronal
health
,
viability
and
number
.
Evidence
suggests
that
NAA
is
a
direct
precursor
for
the
enzymatic
synthesis
of
the
neuron
specific
dipeptide
N-
acetylaspartylglutamate
,
the
most
concentrated
neuropeptide
in
the
human
brain
.
Other
proposed
roles
for
NAA
include
neuronal
osmoregulation
and
axon-glial
signaling
.
We
propose
that
NAA
may
also
be
involved
in
brain
nitrogen
balance
.
Further
research
will
be
required
to
more
fully
understand
the
biochemical
functions
served
by
NAA
in
CNS
development
and
activity
,
and
additional
functions
are
likely
to
be
discovered
.
Diseases
Validation
Diseases presenting
"early postnatal"
symptom
aniridia
canavan disease
omenn syndrome
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