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[Globosides as key players in the pathophysiology of Shiga toxin-associated acute kidney failure and Fabry disease].
[fabry disease]
Globosides
and
their
isomeric
counterparts
isoglobosides
belong
to
the
class
of
neutral
glycosphingolipids
with
an
as
yet
undefined
physiological
function
.
In
the
pathogenesis
of
human
diseases
,
globosides
play
an
important
role
as
cellular
receptors
for
Shiga
toxins
which
are
produced
by
certain
strains
of
S
.
dysenteriae
and
E
.
coli
.
In
order
to
elucidate
the
pathogenesis
of
Shiga
toxin-associated
kidney
failure
,
we
studied
human
kidney
biopsies
and
animal
models
.
Our
work
showed
that
in
patients
suffering
from
Shiga
toxin-elicited
kidney
failure
,
no
complement
activation
could
be
demonstrated
by
immunohistochemical
analysis
of
kidney
biopsies
.
Therefore
,
complement
activation
is
unlikely
to
play
a
major
role
in
mediating
thrombotic
microangiopathy
on
exposure
to
Shiga
toxin
.
Moreover
,
analysis
of
the
human
biopsies
and
of
a
murine
model
of
Shiga
toxin-associated
disease
pinpointed
acute
tubular
damage
as
an
important
and
previously
neglected
contributor
to
acute
kidney
failure
in
patients
infected
with
Shiga
toxin-producing
E
.
coli
.
Furthermore
,
globosides
play
a
decisive
role
in
the
pathogenesis
of
Fabry
disease
which
results
from
a
decreased
or
absent
activity
of
the
lysosomal
enzyme
α-galactosidase
Â
A
.
The
results
on
transgenic
mice
showed
that
in
vital
organs
,
such
as
the
heart
,
kidneys
and
liver
,
it
was
possible
to
revert
the
phenotype
of
Fabry
disease
by
eliminating
the
synthesis
of
globosides
.
This
implicates
that
substrate
reduction
therapy
through
inhibition
of
globosides
might
represent
a
new
therapeutic
option
for
Fabry
disease
,
all
the
more
so
as
globosides
seem
to
be
dispensable
.
Diseases
Validation
Diseases presenting
"toxin-elicited kidney failure"
symptom
fabry disease
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