Everything about Gdp-fucose Protein O-fucosyltransferase 1 totally explained
GDP-fucose protein O-fucosyltransferase 1 (POFUT1) is an
enzyme responsible for adding
fucose sugars in
O linkage to
serine or
threonine residues between the second and third conserved cysteines in
EGF-like repeats on the
Notch protein. The protein is an inverting
glycosyltransferase, which means that the enzyme uses GDP-β-L-fucose as a donor substrate and transfers the fucose in
O linkage to the protein producing fucose-α-
O-serine/threonine.
When the gene for POFUT1 is knocked out, or the expression is decreased to very low levels, all
Notch signaling is destroyed, which means that fucose on Notch is absolutely essential for Notch function. Why this is the case isn't yet well understood.
Almost all glycosyltransferases reside in the
Golgi apparatus. However, POFUT1 as well as the related
enzyme POFUT2 have recently been shown to reside in the
endoplasmic reticulum.
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