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Examination of the mechanism of human brain aspartoacylase through the binding of an intermediate analogue.
[canavan disease]
Canavan
disease
is
a
fatal
neurological
disorder
caused
by
the
malfunctioning
of
a
single
metabolic
enzyme
,
aspartoacylase
,
that
catalyzes
the
deacetylation
of
N-
acetyl-
L-
aspartate
to
produce
L-
aspartate
and
acetate
.
The
structure
of
human
brain
aspartoacylase
has
been
determined
in
complex
with
a
stable
tetrahedral
intermediate
analogue
,
N-
phosphonomethyl-
L-
aspartate
.
This
potent
inhibitor
forms
multiple
interactions
between
each
of
its
heteroatoms
and
the
substrate
binding
groups
arrayed
within
the
active
site
.
The
binding
of
the
catalytic
intermediate
analogue
induces
the
conformational
ordering
of
several
substrate
binding
groups
,
thereby
setting
up
the
active
site
for
catalysis
.
The
highly
ordered
binding
of
this
inhibitor
has
allowed
assignments
to
be
made
for
substrate
binding
groups
and
provides
strong
support
for
a
carboxypeptidase-
type
mechanism
for
the
hydrolysis
of
the
amide
bond
of
the
substrate
,
N-
acetyl-
l-aspartate
.
Diseases
Validation
Diseases presenting
"type mechanism for the hydrolysis of the amide bond"
symptom
canavan disease
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