Article
Structural basis for a human glycosylation disorder caused by mutation of the COG4 gene.
Proceedings of the National Academy of Sciences of the United States of America - 11 Aug 2009
Richardson Brian C, Smith Richard D, Ungar Daniel, Nakamura Ayumi, Jeffrey Philip D, Lupashin Vladimir V, Hughson Frederick M
Abstract excerpt
The proper glycosylation of proteins trafficking through the Golgi apparatus depends upon the conserved oligomeric Golgi (COG) complex. Defects in COG can cause fatal congenital disorders of glycosylation (CDGs) in humans. The recent discovery of a form of CDG, caused in part by a COG4 missense mutation changing Arg 729 to Trp, prompted us to determine the 1.9 A crystal structure of a Cog4 C-terminal fragment....
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