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An improved spectrophotometric assay of pyruvate dehydrogenase in lactate dehydrogenase contaminated mitochondrial preparations from human skeletal muscle.
[pyruvate dehydrogenase deficiency]
In
mitochondria-enriched
preparations
of
human
skeletal
muscle
,
the
measurement
of
pyruvate
dehydrogenase
activity
,
as
determined
by
conventional
spectrophotometric
assay
of
NADH
accumulation
,
is
underestimated
due
to
the
oxidizing
activity
of
the
contaminating
lactate
dehydrogenase
.
Using
a
model
reaction
system
consisting
of
varying
mixtures
of
purified
lactate
and
pyruvate
dehydrogenases
,
we
found
that
the
presence
of
oxamate
,
a
competitive
inhibitor
of
the
lactate
dehydrogenase
,
allowed
the
measurement
of
a
linear
rate
of
pyruvate
dehydrogenase
activity
without
interference
from
lactate
dehydrogenase
.
In
the
presence
of
25
mM
oxamate
,
this
holds
true
up
to
a
ratio
of
30
:
1
for
lactate
to
pyruvate
dehydrogenases
,
respectively
.
A
similar
result
was
obtained
when
using
human
skeletal
muscle
mitochondria
contaminated
by
lactate
dehydrogenase
.
Rates
of
pyruvate
dehydrogenase
activity
ranging
from
50
to
120
nmol
/
min
/
mg
protein
could
be
routinely
measured
in
such
mitochondrial
fractions
.
We
concluded
that
the
use
of
oxamate
allows
a
spectrophotometric
assay
for
pyruvate
dehydrogenase
activity
to
be
utilized
when
screening
for
pyruvate
dehydrogenase
deficiency
in
mitochondria-enriched
preparations
of
human
skeletal
muscle
.
Diseases
Validation
Diseases presenting
"skeletal muscle"
symptom
cadasil
epidermolysis bullosa simplex
esophageal adenocarcinoma
focal myositis
hereditary cerebral hemorrhage with amyloidosis
inclusion body myositis
lamellar ichthyosis
neuralgic amyotrophy
pleomorphic liposarcoma
pyomyositis
pyruvate dehydrogenase deficiency
systemic capillary leak syndrome
well-differentiated liposarcoma
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