NEU1,4 hydrolyze PSAP(195-273):GM3:PE

H2O + PSAP(195-273):GM3:PE → Neu5Ac + PSAP(195-273):LacCer:PE

Reactome

R-HSA-1605724

Compartment

lysosomal lumen

Rhea

None recorded

Catalysed by

• exo-alpha-sialidase activity of NEU1,4 [lysosomal lumen]EC 3.2.1.18

Literature

1. Characterization of human lysosomal neuraminidase defines the molecular basis of the metabolic storage disorder sialidosis Genes Dev, 1996
2. Expression of cDNA encoding the human "protective protein" associated with lysosomal beta-galactosidase and neuraminidase: homology to yeast proteases Cell, 1988
3. A mutation in a mild form of galactosialidosis impairs dimerization of the protective protein and renders it unstable EMBO J, 1991
4. Three-dimensional structure of the human 'protective protein': structure of the precursor form suggests a complex activation mechanism Structure, 1995
5. Neu4, a novel human lysosomal lumen sialidase, confers normal phenotype to sialidosis and galactosialidosis cells J Biol Chem, 2004
6. Prosaposin deficiency: further characterization of the sphingolipid activator protein-deficient sibs. Multiple glycolipid elevations (including lactosylceramidosis), partial enzyme deficiencies and ultrastructure of the skin in this generalized sphingolipid storage disease Hum Genet, 1993