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Structure paper

TitleStructure of human lysosomal acid alpha-glucosidase-a guide for the treatment of Pompe disease.
Journal, issue, pagesNat Commun, Vol. 8, Page 1111-1111, Year 2017
Publish dateApr 8, 2017 (structure data deposition date)
AuthorsRoig-Zamboni, V. / Cobucci-Ponzano, B. / Iacono, R. / Ferrara, M.C. / Germany, S. / Bourne, Y. / Parenti, G. / Moracci, M. / Sulzenbacher, G.
External linksNat Commun / PubMed:29061980
MethodsX-ray diffraction
Resolution1.83 - 2.45 Å
Structure data

PDB-5nn3:
Crystal structure of human lysosomal acid-alpha-glucosidase, GAA
Method: X-RAY DIFFRACTION / Resolution: 1.9 Å

PDB-5nn4:
Crystal structure of human lysosomal acid-alpha-glucosidase, GAA, in complex with N-acetyl-cysteine
Method: X-RAY DIFFRACTION / Resolution: 1.83 Å

PDB-5nn5:
Crystal structure of human lysosomal acid-alpha-glucosidase, GAA, in complex with 1-deoxynojirimycin
Method: X-RAY DIFFRACTION / Resolution: 2 Å

PDB-5nn6:
Crystal structure of human lysosomal acid-alpha-glucosidase, GAA, in complex with N-hydroxyethyl-1-deoxynojirimycin
Method: X-RAY DIFFRACTION / Resolution: 2 Å

PDB-5nn8:
Crystal structure of human lysosomal acid-alpha-glucosidase, GAA, in complex with acarbose
Method: X-RAY DIFFRACTION / Resolution: 2.45 Å

Chemicals

ChemComp-SO4:
SULFATE ION

ChemComp-CL:
Unknown entry

ChemComp-PGE:
TRIETHYLENE GLYCOL

ChemComp-PEG:
DI(HYDROXYETHYL)ETHER

ChemComp-EDO:
1,2-ETHANEDIOL

ChemComp-HOH:
WATER

ChemComp-SC2:
N-ACETYL-L-CYSTEINE / medication*YM

ChemComp-GOL:
GLYCEROL

ChemComp-NOJ:
1-DEOXYNOJIRIMYCIN / inhibitor*YM

ChemComp-MIG:
(2R,3R,4R,5S)-1-(2-hydroxyethyl)-2-(hydroxymethyl)piperidine-3,4,5-triol

Source
  • homo sapiens (human)
KeywordsHYDROLASE / glycoside hydrolase / lysosome / glycogen catabolism / Pompe disease

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