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:3.1.6.4 (
chondroitinase
)
2,039
document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)
Three acidic glycosidases: beta-galactosidase (beta-GAL, EC 3.2.1.23), alpha-neuraminidase (NEUR, sialidase, EC 3.2.1.18), N-acetylaminogalacto-6-sulfate sulfatase (GALNS,
EC 3.1.6.4
) and serine carboxypepidase
cathepsin A
(EC 3.4.16.1) form a functional high molecular weight complex in the lysosomes. The major constituent of this complex is
cathepsin A
, the so-called "lysosomal protective protein" (PPCA). By forming a multienzyme complex, it protects the glycosidases from rapid intralysosomal proteolysis, and it is also required for the intracellular sorting and proteolytic processing of their precursors. In man, a deficiency of
cathepsin A
leads to a combined deficiency of beta-GAL and NEUR activities, called "galactosialidosis". Multiple mutations identified in the
cathepsin A
gene are the molecular basis of this lysosomal storage disease. This review describes the structural organization of the lysosomal high molecular weight multienzyme complex and the importance of the protective protein/
cathepsin A
in physiology and pathology.
...
PMID:Lysosomal high molecular weight multienzyme complex. 1265 52
