α-galactosidase transgenic mouse:: Heterogeneous gene expression and posttranslational glycosylation in tissues

被引：11

作者：

Ishii, S

Kase, R

Sakuraba, H

Taya, C

Yonekawa, H

Okumiya, T

Matsuda, Y

Mannen, K

Takeshita, M

Suzuki, Y

机构：

[1] Tokyo Metropolitan Inst Med Sci, Clin Genet, Bunkyo Ku, Tokyo 113, Japan

[2] Tokyo Metropolitan Inst Med Sci, Lab Anim Sci, Bunkyo Ku, Tokyo 113, Japan

[3] Usuki Bio Res Ctr, Oita 875, Japan

[4] Kochi Med Sch, Dept Lab Med, Nankoku, Kochi 783, Japan

[5] Nagoya Univ, Sch Agr Sci, Lab Anim Genet, Chikusa Ku, Nagoya, Aichi 46401, Japan

[6] Oita Med Univ, Sch Med, Anim Lab Ctr, Oita 87955, Japan

[7] Oita Med Univ, Sch Med, Dept Biochem, Oita 87955, Japan

来源：

GLYCOCONJUGATE JOURNAL | 1998年 / 15卷 / 06期

关键词：

alpha-galactosidase; Fabry disease; transgenic mouse; glycosylation;

D O I：

10.1023/A:1006915926732

中图分类号：

Q5 [生物化学]; Q7 [分子生物学];

学科分类号：

071010 ; 081704 ;

摘要：

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.

引用

页码：591 / 594

页数：4

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