title: Alpha-galactosidase transgenic mouse: heterogeneous gene expression and posttranslational glycosylation in tissues.
abstract: We produced six transgenic mouse lines expressing human alpha-galactosidase (alpha-Gal) in order to evaluate its posttranslational modification. Among them, serum alpha-Gal activity increased 3000-fold in two transgenic mouse lines (TgN2 and TgN51), as compared to that in non-transgenic lines. The heart and liver of the TgN2 mouse expressed a high amount of transcript as well as high alpha-Gal activity. Its gene products in the heart and kidney were sensitive to endoglycosidase H digestion, but those in the spleen and liver were largely resistant. Glycopeptidase F treatment confirmed an identical molecular mass for the peptide moiety of the enzyme. We concluded that heterogeneous molecular mass of the gene products was caused by different degrees of posttranslational glycosylation in murine tissues.
authors.affiliation: Usuki Bio Research Center, Oita, Japan.
authors.forename: S
authors.initials: S
authors.lastname: Ishii
authors.forename: R
authors.initials: R
authors.lastname: Kase
authors.forename: H
authors.initials: H
authors.lastname: Sakuraba
authors.forename: C
authors.initials: C
authors.lastname: Taya
authors.forename: H
authors.initials: H
authors.lastname: Yonekawa
authors.forename: T
authors.initials: T
authors.lastname: Okumiya
authors.forename: Y
authors.initials: Y
authors.lastname: Matsuda
authors.forename: K
authors.initials: K
authors.lastname: Mannen
authors.forename: M
authors.initials: M
authors.lastname: Takeshita
authors.forename: Y
authors.initials: Y
authors.lastname: Suzuki
mesh_terms: D000818:Animals; D015971:Gene Expression Regulation, Enzymologic; D006031:Glycosylation; D006801:Humans; D007668:Kidney / Q000201:enzymology; D008099:Liver / Q000201:enzymology; D051379:Mice; D008822:Mice, Transgenic; D009206:Myocardium / Q000201:enzymology; D011499:Protein Processing, Post-Translational; D013154:Spleen / Q000201:enzymology; D000519:alpha-Galactosidase / Q000097:blood / Q000235:genetics* / Q000378:metabolism
chemical_list: D000519:alpha-Galactosidase