1KTC

The Structure of alpha-N-Acetylgalactosaminidase

1KTC の概要

• エントリーDOI: 10.2210/pdb1ktc/pdb
• 関連するPDBエントリー: 1KTB
• 分子名称: alpha-N-acetylgalactosaminidase, 2-acetamido-2-deoxy-beta-D-glucopyranose-(1-4)-2-acetamido-2-deoxy-beta-D-glucopyranose, 2-acetamido-2-deoxy-beta-D-glucopyranose, ... (8 entities in total)
• 機能のキーワード: glycoprotein, (beta/alpha)8 barrel, protein-ligand complex, hydrolase
• 由来する生物種: Gallus gallus (chicken)
• タンパク質・核酸の鎖数: 1
• 化学式量合計: 48194.50

構造登録者

Garman, S.C.,Hannick, L.,Zhu, A.,Garboczi, D.N. (登録日: 2002-01-15, 公開日: 2002-03-15, 最終更新日: 2024-10-30)

主引用文献

Garman, S.C.,Hannick, L.,Zhu, A.,Garboczi, D.N.
The 1.9 A structure of alpha-N-acetylgalactosaminidase: molecular basis of glycosidase deficiency diseases.
Structure, 10:425-434, 2002

PubMed Abstract: In the lysosome, glycosidases degrade glycolipids, glycoproteins, and oligosaccharides. Mutations in glycosidases cause disorders characterized by the deposition of undegraded carbohydrates. Schindler and Fabry diseases are caused by the incomplete degradation of carbohydrates with terminal alpha-N-acetylgalactosamine and alpha-galactose, respectively. Here we present the X-ray structure of alpha-N-acetylgalactosaminidase (alpha-NAGAL), the glycosidase that removes alpha-N-acetylgalactosamine, and the structure with bound ligand. The active site residues of alpha-NAGAL are conserved in the closely related enzyme a-galactosidase A (alpha-GAL). The structure demonstrates the catalytic mechanisms of both enzymes and reveals the structural basis of mutations causing Schindler and Fabry diseases. As alpha-NAGAL and alpha-GAL produce type O "universal donor" blood from type A and type B blood, the alpha-NAGAL structure will aid in the engineering of improved enzymes for blood conversion.

PubMed: 12005440
DOI: 10.1016/S0969-2126(02)00726-8

実験手法

X-RAY DIFFRACTION (2.4 Å)