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Ceramidase
Ceramidase
is an
enzyme
which
cleaves
fatty acids
from
ceramide
,
producing
sphingosine
which in turn is
phosphorylated
by a
sphingosine kinase
to form
sphingosine-1-phosphate
.
Function
Ceramide
, SPH, and S1P are
bioactive
lipids
that mediate
cell proliferation
,
differentiation
,
apoptosis
,
adhesion
, and
migration
. Presently, 7
human
ceramidases encoded by 7
distinct
genes have been cloned:
acid ceramidase
–
cell survival
neutral
ceramidase –
protective
against
inflammatory
cytokines
alkaline
ceramidase 1 –
mediating
cell differentiation
by
controlling
the
generation
of SPH and S1P
alkaline ceramidase 2 – important for cell
proliferation
and
survival
alkaline ceramidase 3
Clinical significance
A
deficiency
in
ASAH1
is associated with
Farber disease
.