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Morphological analysis of corneal opacity in Shiba dog with GM1 gangliosidosis.
[gm1 gangliosidosis]
GM
1
gangliosidosis
is
one
of
the
inherited
metabolic
lysosomal
storage
disorders
characterized
by
neurological
symptoms
caused
by
beta
-galactosidase
deficiency
and
consequent
accumulation
of
GM
1
ganglioside
in
neuronal
cells
.
Shiba
dogs
affected
with
GM
1
gangliosidosis
have
been
found
to
suffer
from
corneal
opacity
.
In
our
morphological
analysis
,
keratocyte
enlargement
was
induced
by
abnormal
intracellular
accumulation
of
neutral
carbohydrates
,
resulting
in
the
loss
of
normal
arrangement
of
collagen
fibrils
in
the
opaque
cornea
was
found
to
be
associated
with
the
disorder
.
We
therefore
conclude
that
corneal
opacity
in
this
Shiba
dog
with
GM
1
gangliosidosis
may
be
caused
by
neutral
carbohydrate
accumulation
in
lysosomes
,
swelling
and
dysfunction
of
keratocytes
,
and
subsequent
irregular
arrangement
of
collagen
fibrils
in
the
corneal
proper
substance
.
Diseases
Validation
Diseases presenting
"neutral carbohydrate accumulation in lysosomes"
symptom
gm1 gangliosidosis
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