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Co-administration of creatine plus pyruvate prevents the effects of phenylalanine administration to female rats during pregnancy and lactation on enzymes activity of energy metabolism in cerebral cortex and hippocampus of the offspring.
[phenylketonuria]
Phenylketonuria
(
PKU
)
is
the
most
frequent
inborn
error
of
metabolism
.
It
is
caused
by
deficiency
in
the
activity
of
phenylalanine
hydroxylase
,
leading
to
accumulation
of
phenylalanine
and
its
metabolites
.
Untreated
maternal
PKU
or
hyperphenylalaninemia
may
result
in
nonphenylketonuric
offspring
with
low
birth
weight
and
neonatal
sequelae
,
especially
microcephaly
and
intellectual
disability
.
The
mechanisms
underlying
the
neuropathology
of
brain
injury
in
maternal
PKU
syndrome
are
poorly
understood
.
In
the
present
study
,
we
evaluated
the
possible
preventive
effect
of
the
co
-administration
of
creatine
plus
pyruvate
on
the
effects
elicited
by
phenylalanine
administration
to
female
Wistar
rats
during
pregnancy
and
lactation
on
some
enzymes
involved
in
the
phosphoryltransfer
network
in
the
brain
cortex
and
hippocampus
of
the
offspring
at
21
Â
days
of
age
.
Phenylalanine
administration
provoked
diminution
of
body
,
brain
cortex
an
hippocampus
weight
and
decrease
of
adenylate
kinase
,
mitochondrial
and
cytosolic
creatine
kinase
activities
.
Co
-administration
of
creatine
plus
pyruvate
was
effective
in
the
prevention
of
those
alterations
provoked
by
phenylalanine
,
suggesting
that
altered
energy
metabolism
may
be
important
in
the
pathophysiology
of
maternal
PKU
.
If
these
alterations
also
occur
in
maternal
PKU
,
it
is
possible
that
pyruvate
and
creatine
supplementation
to
the
phenylalanine-
restricted
diet
might
be
beneficial
to
phenylketonuric
mothers
.
Diseases
Validation
Diseases presenting
"hyperphenylalaninemia"
symptom
classical phenylketonuria
homocystinuria without methylmalonic aciduria
phenylketonuria
This symptom has already been validated
