5nn6

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Crystal structure of human lysosomal acid-alpha-glucosidase, GAA, in complex with N-hydroxyethyl-1-deoxynojirimycinCrystal structure of human lysosomal acid-alpha-glucosidase, GAA, in complex with N-hydroxyethyl-1-deoxynojirimycin

Structural highlights

5nn6 is a 1 chain structure. Full crystallographic information is available from OCA. For a guided tour on the structure components use FirstGlance.
Ligands:, , , , , , , ,
NonStd Res:
Activity:Alpha-glucosidase, with EC number 3.2.1.20
Resources:FirstGlance, OCA, PDBe, RCSB, PDBsum, ProSAT

Disease

[LYAG_HUMAN] Glycogen storage disease due to acid maltase deficiency, infantile onset;Glycogen storage disease due to acid maltase deficiency, juvenile onset;Glycogen storage disease due to acid maltase deficiency, adult onset. The disease is caused by mutations affecting the gene represented in this entry.

Function

[LYAG_HUMAN] Essential for the degradation of glygogen to glucose in lysosomes.

5nn6, resolution 2.00Å

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OCA