Definition of Tay-Sachs Disease
Tay´-Sachs Disease
n.
1.
(Med.)
A
rare
hereditary
disease
affecting
lipid
metabolism
in
humans
,
due
to
a
deficiency
of
hexosaminidase
.
It
occurs
in
infants
and
children
,
and
causes
death
before
the
onset
of
adulthood
.
It
occurs
most
commonly
of
people
of
Jewish
origin
from
easter
Europe
.
It
is
characterized
by
accumulation
of
lipids
in
nervous
tissue
,
causes
a
red
spot
on
the
retina
,
and
eventual
blindness
and
paralysis
before
death
.
Related Words
autosomal
recessive
defect
,
autosomal
recessive
disease
,
infantile
amaurotic
idiocy
,
lipidosis
,
monogenic
disease
,
monogenic
disorder
,
Sachs
disease
,
Tay-Sachs
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