1IVY

PHYSIOLOGICAL DIMER HPP PRECURSOR

Summary for 1IVY

• Entry DOI: 10.2210/pdb1ivy/pdb
• Descriptor: HUMAN PROTECTIVE PROTEIN, 2-acetamido-2-deoxy-alpha-D-glucopyranose-(1-4)-2-acetamido-2-deoxy-beta-D-glucopyranose, 2-acetamido-2-deoxy-beta-D-glucopyranose-(1-4)-2-acetamido-2-deoxy-beta-D-glucopyranose, ... (5 entities in total)
• Functional Keywords: carboxypeptidase, serine carboxypeptidase, protective protein, glycoprotein, zymogen
• Biological source: Homo sapiens (human)
• Total number of polymer chains: 2
• Total formula weight: 104263.23

Authors

Rudenko, G.,Bonten, E.,D'Azzo, A.,Hol, W.G.J. (deposition date: 1996-06-12, release date: 1997-04-21, Last modification date: 2024-10-23)

Primary citation

Rudenko, G.,Bonten, E.,d'Azzo, A.,Hol, W.G.
Three-dimensional structure of the human 'protective protein': structure of the precursor form suggests a complex activation mechanism.
Structure, 3:1249-1259, 1995

PubMed Abstract: The human 'protective protein' (HPP) forms a multi-enzyme complex with beta-galactosidase and neuraminidase in the lysosomes, protecting these two glycosidases from degradation. In humans, deficiency of HPP leads to the lysosomal storage disease galactosialidosis. Proteolytic cleavage of the precursor form of HPP involves removal of a 2 kDa excision peptide and results in a carboxypeptidase activity. The physiological relevance of this activity is, as yet, unknown.

PubMed: 8591035
DOI: 10.1016/S0969-2126(01)00260-X

Experimental method

X-RAY DIFFRACTION (2.2 Å)