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Baicalein 5,6,7-trimethyl ether activates peroxisomal but not mitochondrial fatty acid beta-oxidation.
[zellweger syndrome]
Recently
,
we
reported
that
baicalein
5
,
6
,
7
-
trimethyl
ether
(
BTM
)
,
a
flavonoid
,
is
capable
of
activating
fatty
acid
beta
-oxidation
in
X-
linked
adrenoleukodystrophy
(
X-
ALD
)
fibroblasts
(
FEBS
Lett
.
2005
;
579
:
409
-
414
)
.
The
objective
of
this
study
was
to
clarify
whether
BTM
activates
peroxisomal
and
/
or
mitochondrial
fatty
acid
beta
-oxidation
.
We
first
analysed
the
effect
of
BTM
on
fatty
acid
beta
-oxidation
in
fibroblasts
derived
from
healthy
controls
as
well
as
patients
with
X-
ALD
,
mitochondrial
carnitine-acylcarnitine
translocase
(
CACT
)
deficiency
,
and
peroxisome
biogenesis
disorder
,
Zellweger
syndrome
.
Lignoceric
acid
(
C
(
24
:
0
)
)
beta
-oxidation
in
the
fibroblasts
was
stimulated
by
treatment
with
BTM
,
except
for
Zellweger
fibroblasts
.
In
contrasts
,
palmitic
acid
(
C
(
16
:
0
)
)
beta
-oxidation
was
increased
(
2
.
8
-
fold
)
only
in
CACT-
deficient
fibroblasts
.
In
U
87
glioblastoma
cells
,
C
(
24
:
0
)
beta
-oxidation
was
also
activated
by
treatment
with
BTM
but
C
(
16
:
0
)
beta
-oxidation
was
not
.
The
C
(
16
:
0
)
beta
-oxidation
was
,
however
,
significantly
increased
in
the
presence
of
2
-
[
5
-
(
4
-
chlorophenyl
)
pentyl
]
oxirane-
2
-
carboxylate
(
POCA
)
,
a
carnitine
palmitoyltransferase
I
inhibitor
.
These
results
indicate
that
BTM
activates
peroxisomal
but
not
mitochondrial
fatty
acid
beta
-oxidation
.
In
addition
,
we
found
that
BTM
did
not
upregulate
the
expression
of
ABCD
2
/
ALDR
,
ABCD
3
/
PMP
70
,
ACOX
1
and
FATP
4
genes
but
slightly
increased
ACSVL
1
gene
expression
.
Diseases
Validation
Diseases presenting
"glioblastoma"
symptom
alexander disease
canavan disease
cholangiocarcinoma
erdheim-chester disease
homocystinuria without methylmalonic aciduria
kallmann syndrome
proteus syndrome
severe combined immunodeficiency
zellweger syndrome
This symptom has already been validated