1IVY

PHYSIOLOGICAL DIMER HPP PRECURSOR

1IVY の概要

• エントリーDOI: 10.2210/pdb1ivy/pdb
• 分子名称: 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)
• 機能のキーワード: carboxypeptidase, serine carboxypeptidase, protective protein, glycoprotein, zymogen
• 由来する生物種: Homo sapiens (human)
• タンパク質・核酸の鎖数: 2
• 化学式量合計: 104263.23

構造登録者

Rudenko, G.,Bonten, E.,D'Azzo, A.,Hol, W.G.J. (登録日: 1996-06-12, 公開日: 1997-04-21, 最終更新日: 2024-10-23)

主引用文献

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

実験手法

X-RAY DIFFRACTION (2.2 Å)