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Preservation of high phenylalanine ammonia lyase activities in roots of Japanese Striped corn: a potential oral therapeutic to treat phenylketonuria.
[phenylketonuria]
Phenylketonuria
(
PKU
)
is
an
inherited
metabolic
disorder
caused
by
deficient
phenylalanine
hydroxylase
(
PAH
)
activity
,
the
enzyme
responsible
for
the
disposal
of
excess
amounts
of
the
essential
amino
acid
phenylalanine
(
Phe
)
.
Phenylalanine
ammonia-lyase
(
PAL
,
EC
4
.
3
.
1
.
5
)
has
potential
to
serve
as
an
enzyme
substitution
therapy
for
this
human
genetic
disease
.
Using
7
-
day
-old
Japanese
Striped
corn
seedlings
(
Japonica
Striped
maize
,
Zea
mays
L
.
cv
.
japonica
)
that
contain
high
activities
of
PAL
,
we
investigated
a
number
of
methods
to
preserve
the
roots
as
an
intact
food
and
for
long
-term
storage
.
The
cryoprotectant
effects
of
maple
syrup
and
other
edible
sugars
(
mono-
and
oligosaccharides
)
were
evaluated
.
Following
thawing
,
the
preserved
roots
were
then
examined
to
determine
whether
the
rigid
plant
cell
walls
could
protect
the
PAL
enzyme
from
proteolysis
during
simulated
(
in
vitro
)
digestion
comprised
of
gastric
and
intestinal
phases
.
While
several
treatments
led
to
retention
of
PAL
activity
during
freezing
,
upon
thawing
and
in
vitro
digestion
,
root
tissues
that
had
been
previously
frozen
in
the
presence
of
maple
syrup
exhibited
the
highest
residual
PAL
activities
(
∼
50
%
of
the
initial
enzyme
activity
)
,
in
marked
contrast
to
all
of
the
treatments
using
other
edible
sugars
.
The
structural
integrity
of
the
root
cells
,
and
the
stability
of
the
functional
PAL
tetramer
were
also
preserved
with
the
maple
syrup
protocol
.
These
results
have
significance
for
the
formulation
of
oral
enzyme
/
protein
therapeutics
.
When
plant
tissues
are
adequately
preserved
,
the
rigid
cell
walls
constitute
a
protective
barrier
even
under
harsh
(
e
.
g
.
gastrointestinal
-like
)
conditions
.
Diseases
Validation
Diseases presenting
"human genetic disease"
symptom
familial hypocalciuric hypercalcemia
junctional epidermolysis bullosa
phenylketonuria
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