Abstract
Defects in protein glycosylation can have a dramatic impact on eukaryotic cells and is associated with mental and developmental pathologies in humans. The studies outlined below illustrate how a basic biochemical problem in the mechanisms of protein glycosylation, specifically substrate transporters of nucleotide sugars, including ATP and 3'-phosphoadenyl-5'-phosphosulfate (PAPS), in the membrane of the Golgi apparatus and endoplasmic reticulum, expanded into diverse biological systems from mammals, including humans, to yeast, roundworms, and protozoa. Using these diverse model systems allowed my colleagues and me to answer fundamental biological questions that enabled us to formulate far-reaching hypotheses and expanded our knowledge of human diseases caused by malfunctions in the metabolic processes involved.
Highlights
Defects in protein glycosylation can have a dramatic impact on eukaryotic cells and is associated with mental and developmental pathologies in humans
When we incubated cells grown in monolayers with nucleotide sugars, in the presence of incubation buffers, we found that virtually all nucleotide sugars were degraded, and free radioactive sugar was found in the incubation medium
In collaboration with Stu Swiedler’s group, we showed that the enzyme had the above dual activity, that it functioned as a monomer in the Golgi membrane, and that its active site was on the C terminus
Summary
Defects in protein glycosylation can have a dramatic impact on eukaryotic cells and is associated with mental and developmental pathologies in humans. The studies outlined below illustrate how a basic biochemical problem in the mechanisms of protein glycosylation, substrate transporters of nucleotide sugars, including ATP and 3-phosphoadenyl-5phosphosulfate (PAPS), in the membrane of the Golgi apparatus and endoplasmic reticulum, expanded into diverse biological systems from mammals, including humans, to yeast, roundworms, and protozoa. Using these diverse model systems allowed my colleagues and me to answer fundamental biological questions that enabled us to formulate far-reaching hypotheses and expanded our knowledge of human diseases caused by malfunctions in the metabolic processes involved
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