MOLECULAR BIOLOGY OF ASPARAGINE LINKED GLYCOSYLATION
Project Number2R01GM038545-09
Contact PI/Project LeaderLEHRMAN, MARK
Awardee OrganizationUT SOUTHWESTERN MEDICAL CENTER
Description
Abstract Text
The goal of this proposal is to understand the structure and function of
hamster GlcNAc-1-P transferase (GPT). GPT catalyzes the transfer of
GlcNAc-1-P from UDP-GlcNAc to dolichol-P to form GlcNAc-P-P-Dolichol.
GlcNAc-PP-dolichol is then converted into oligosaccharide-PP-dolichol
(with 14 sugars), the immediate precursor for N-linked glycans in
eukaryotes. N-linked glycans are important in a broad range of functions,
such as cell adhesion, organellar trafficking, and protein folding, and
have been implicated in cancer and human diseases such as HEMPAS, CGDS,
and I-cell disease. Recent amino acid sequence analyses have linked
hamster GPT with a family of similar eukaryotic and prokaryotic sugar-1-P
transferases which use UDP-GlcNAc or UDP-MurNAc-pentapeptide as a donor
and a polyisoprenyl phosphate as an acceptor.
It is essential that GPT activity be regulated within a narrowly defined
window. Too little GPT activity prevents sufficient quantities of N-linked
glycans from being produced. Too much GPT activity causes excessive
competition between GlcNAc-P-P-dolichol synthesis and reactions
synthesizing mannose-P-dolichol (MPD) and glucose-P-dolichol (GPD).
Insufficient MPD and GPD results in abnormal N-linked glycosylation and
defective glycosylphosphatidylinositol (GPI) anchor synthesis.
Furthermore, recent data indicate that binding of unfolded proteins to the
ER chaperone calnexin, which specifically recognizes Glc1Man9GlcNAc2,
would be inhibited.
Five specific aims are proposed to address the following questions about
hamster GPT: (1) What are the functions of the conserved sequences found
in members of the UDP-GlcNAc/MurNAc transferase family? (2) What
determines specificity for different sugar donors and lipid acceptors in
the UDP-GlcNAc/MurNAc family of glycosyltransferases? (3) Rather than
mediating bind of dolichol, does the "potential dolichol recognition
sequence" (PDRS) promote a complex of enzymes which act early in the
dolichol pathway? (4) How is GPT stabilized by its sequence-specific,
bipartite C-terminus? (5) What is the topological orientation of GPT in
the ER membrane? The answers to these questions will not only provide new
fundamental information about GPT and its role in N-linked glycosylation,
but will also have direct implications for a number of structurally
related proteins.
No Sub Projects information available for 2R01GM038545-09
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