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| Alpha-mannosidase-II deficiency results in dyserythropoiesis and unveils an alternate pathway in oligosaccharide biosynthesis |
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| Author(s): Chui D, OhEda M, Liao YF, Panneerselvam K, Lal A, Marek KW, Freeze HH, Moremen KW, Fukuda MN, Marth JD |
| Source: CELL Volume: 90 Issue: 1 Pages: 157-167 Published: JUL 11 1997 |
| Times Cited: 119 References: 56 |
| Abstract: Alpha-mannosidase-II (alpha M-II) catalyzes the first committed step in the biosynthesis of complex asparagine-linked (N-linked) oligosaccharides (N-glycans). Genetic deficiency of alpha M-II should abolish complex N-glycan production as reportedly does inhibition of alpha M-II by swainsonine. We find that mice lacking a functional alpha M-II gene develop a dyserythropoietic anemia concurrent with loss of erythrocyte complex N-glycans. Unexpectedly, nonerythroid cell types continued to produce complex N-glycans by an alternate pathway comprising a distinct alpha-mannosidase. These studies reveal cell-type-specific variations in N-linked oligosaccharide biosynthesis and an essential role for alpha M-II in the formation of erythroid complex N-glycans. alpha M-II deficiency elicits a phenotype in mice that correlates with human congenital dyserythropoietic anemia type II. |
| Document Type: Article |
| Language: English |
Addresses:
1. UNIV CALIF SAN DIEGO, HOWARD HUGHES MED INST, LA JOLLA, CA 92093 USA
2. UNIV CALIF SAN DIEGO, DIV CELLULAR & MOL MED, LA JOLLA, CA 92093 USA
3. BURNHAM INST, LA JOLLA, CA 92037 USA
4. UNIV GEORGIA, DEPT BIOCHEM, ATHENS, GA 30602 USA |
| Publisher: CELL PRESS, 1050 MASSACHUSETTES AVE, CIRCULATION DEPT, CAMBRIDGE, MA 02138 |
| Subject Category: Biochemistry & Molecular Biology; Cell Biology |
| IDS Number: XL362 |
| ISSN: 0092-8674 |
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