title: Crystallization and preliminary X-ray analysis of recombinant human acid beta-glucocerebrosidase, a treatment for Gaucher's disease.
abstract: Acid beta-glucocerebrosidase (N-acylsphingosyl-1-O-beta-D-glucoside:glucohydrolase) is a lysosomal glycoprotein that catalyzes the hydrolysis of the glycolipid glucocerebroside to glucose and ceramide. Inadequate levels of this enzyme underly the pathophysiology of Gaucher's disease. Cerezyme (Genzyme Corporation, Cambridge, MA, USA) is a partially deglycosylated form of recombinant human acid beta-glucocerebrosidase that is used in the treatment of Gaucher patients. Although acid beta-glucocerebrosidase belongs to a large family of glycosidases, relatively little is known regarding its structural biology. Here, the crystallization and the initial diffraction analysis of Cerezyme are reported. The crystals are C-centered orthorhombic, with unit-cell parameters a = 285.0, b = 110.2, c = 91.7 A. A 99.9% complete data set has been collected to 2.75 A with an R(sym) of 8.8%.
authors.affiliation: NASA Laboratory for Structural Biology, Code SD46, NASA MSFC, Huntsville, AL 35812, USA.
authors.forename: Dana
authors.initials: D
authors.lastname: Roeber
authors.forename: Aniruddha
authors.initials: A
authors.lastname: Achari
authors.forename: Partha
authors.initials: P
authors.lastname: Manavalan
authors.forename: Tim
authors.initials: T
authors.lastname: Edmunds
authors.forename: David L
authors.initials: DL
authors.lastname: Scott
mesh_terms: D003460:Crystallization; D018360:Crystallography, X-Ray; D005776:Gaucher Disease / Q000188:drug therapy; D005962:Glucosylceramidase / Q000737:chemistry*; D006801:Humans; D011994:Recombinant Proteins / Q000737:chemistry
chemical_list: D011994:Recombinant Proteins; D005962:Glucosylceramidase; C090568:imiglucerase