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Involvement of corneodesmosome degradation and lamellar granule transportation in the desquamation process.
[lamellar ichthyosis]
Desquamation
in
the
mammalian
skin
is
a
well-balanced
process
of
producing
corneocytes
and
shedding
them
from
the
surface
of
the
skin
.
The
corneodesmosome
,
which
is
a
modified
desmosome
,
is
the
main
adhesive
structure
in
the
cornified
cell
layer
.
The
major
extracellular
constituents
of
corneodesmosomes
are
desmoglein
1
,
desmocollin
1
,
and
corneodesmosin
.
Proteases
involved
in
the
degradation
of
corneodesmosomes
and
their
inhibitors
are
secreted
from
lamellar
granules
in
the
granular
cell
layer
.
Genetic
defects
in
corneodesmosin
and
protease
inhibitors
result
in
accelerated
desquamation
and
severe
barrier
impairment
.
Abnormalities
in
transportation
and
secretion
of
lamellar
granules
underlie
ichthyosis
seen
in
certain
human
diseases
.
