-
fatty tissue structures.
Important lysosomal enzymes, such as acid sphingomyelinase, may be
inhibited by
lysosomally ac****ulated drugs. Such compounds...
-
Lysosomal storage diseases (LSDs; /ˌlaɪsəˈsoʊməl/) are a
group of over 70 rare
inherited metabolic disorders that
result from
defects in
lysosomal function...
-
Lysosomal acid
lipase deficiency (LAL
deficiency or LAL-D) or
Wolman Disease, is an
autosomal recessive inborn error of
metabolism that
results in the...
- Alpha-mannosidosis is a
lysosomal storage disorder,
first described by
Swedish physician Okerman in 1967. In
humans it is
known to be
caused by an autosomal...
- syndrome, or
mucopolysaccharidosis type II (MPS II), is a rare
inherited lysosomal storage disease in
which large sugar molecules called glycosaminoglycans...
-
following chemical reaction:
Hydrolysis of a gamma-glutamyl bond This
lysosomal or secreted, thiol-dependent peptidase, most
active at
acidic pH. In humans...
-
receptors bind
newly synthesized lysosomal hydrolases in the trans-Golgi
network (TGN) and
deliver them to pre-
lysosomal compartments.
There are two different...
- are
stored in the
lysosomal membrane,
which also
house lysosomal membrane glycoproteins. LAMP1 and LAMP2 make up
about 50% of
lysosomal membrane glycoproteins...
-
autoimmune disease. This
includes deficient phagocytic activity,
impaired lysosomal degradation, and
scant serum components in
addition to
increased apoptosis...
- an ac****ulation of
glycogen in the
lysosome due to a
deficiency of the
lysosomal acid alpha-glucosidase
enzyme (GAA). The
inability to
break down glycogen...
