Reactome | FUT1 transfers Fuc to Type 2 chains to form H antigen-RBC

FUT1 transfers Fuc to Type 2 chains to form H antigen-RBC

Stable Identifier

R-HSA-9033949

Type

Reaction [transition]

Species

Homo sapiens

Compartment

Golgi lumen, Golgi membrane

ReviewStatus

5/5

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FUT1 transfers Fuc to Type 2 chains to form H antigen-RBC

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The H antigen is formed with the addition of a fucose (Fuc) sugar onto one of two precursor oligosaccharide sequences, Type 1 (RBCs) or Type 2 (secreted) chains. The FUT1 gene (aka H gene) found in hematopoietic cells produces galactoside 2-α-L-fucosyltransferase 1 (FUT1 aka α-1,2-fucosyltransferase 1) which mediates the transfer of a fucose (Fuc) sugar to the galactose (Gal) sugar of the Type 2 chain precursor Gal-β1,4-GlcNAc-β1,3-Gal-R (where R is a glycosphingolipid) to form the H antigen (Larsen et al. 1990). This is an essential step for subsequent formation of A and B antigens. Mutations that inactivate the FUT1 gene can result in the 'Bombay phenotype' where no A, B or H antigens are produced on RBCs (Koda et al 1997, Kaneko et al. 1997).

Literature References

PubMed ID	Title	Journal	Year
9226185	Wide variety of point mutations in the H gene of Bombay and para-Bombay individuals that inactivate H enzyme Kaneko, M, Nishihara, S, Shinya, N, Kudo, T, Iwasaki, H, Seno, T, Okubo, Y, Narimatsu, H	Blood	1997
2118655	Molecular cloning, sequence, and expression of a human GDP-L-fucose:beta-D-galactoside 2-alpha-L-fucosyltransferase cDNA that can form the H blood group antigen Larsen, RD, Ernst, LK, Nair, RP, Lowe, JB	Proc. Natl. Acad. Sci. U.S.A.	1990
9299444	Missense mutation of FUT1 and deletion of FUT2 are responsible for Indian Bombay phenotype of ABO blood group system Koda, Y, Soejima, M, Johnson, PH, Smart, E, Kimura, H	Biochem. Biophys. Res. Commun.	1997