Gene/Protein
Disease
Symptom
Drug
Enzyme
Compound
Pivot Concepts:
Gene/Protein
Disease
Symptom
Drug
Enzyme
Compound
Target Concepts:
Gene/Protein
Disease
Symptom
Drug
Enzyme
Compound
Query: EC:3.4.23.5 (
cathepsin D
)
4,130
document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)
The distribution of
cathepsin D
, an acidic endopeptidase, was localized by immunocytochemistry in human skeletal muscle obtained from 34 persons with a variety of neuromuscular disorders. Normal human skeletal muscle contained small amounts of
cathepsin D
, all of which was found close to the sarcolemmal membrane. Immunoreactive
cathepsin D
was present in the cytoplasm of many infiltrating phagocytic cells and was increased in skeletal muscle fibers from patients with muscular dystrophies, inflammatory myopathies, rhabdomyolysis,
acid maltase deficiency
, and neurogenic atrophy. In cases of Duchenne type muscular dystrophy, the increase in
cathepsin D
was especially prominent in small regenerating fibers, in which it was visualized at the ultrastructural level in lysosome-like organelles and extralysosomal locations. The function of
cathepsin D
in skeletal muscle is unclear, but the present findings suggest a possible role in muscle regeneration and repair. Such a role would necessitate careful selection of drugs which interfere with proteolytic activity if they are to be used as therapeutic agents in treating neuromuscular diseases.
...
PMID:Immunocytochemical studies of cathepsin D in human skeletal muscle. 633 8
Cultured human skin fibroblasts from control persons and from patients with the generalized and late-onset forms of
Pompe's disease
were labelled with radioactive leucine and the incorporation of radioactivity into acid alpha-glucosidase and
cathepsin D
was analysed by immunoprecipitation, gel electrophoresis and fluorography. When the labelling was carried out for 6-12 h in the presence of NH4Cl, the labelling of secreted alpha-glucosidase relative to that of secreted
cathepsin D
in fibroblasts from patients with the late-onset form of
Pompe's disease
was less than 15% of that in fibroblasts from control persons. However, when the fibroblasts were labelled for less than 1 h, the relative rate of incorporation of radioactivity into acid alpha-glucosidase was rather similar in the two types of fibroblasts. In fibroblasts from patients with the generalized form of
Pompe's disease
no incorporation of radioactivity into acid alpha-glucosidase could be detected.
...
PMID:Biosynthesis of acid alpha-glucosidase in late-onset forms of glycogenosis type II (Pompe's disease). 676 Nov 45
