Reactome | B4GALT7 transfers Gal group to xylosyl-unit of the tetrasaccharide linker

B4GALT7 transfers Gal group to xylosyl-unit of the tetrasaccharide linker

Stable Identifier

R-HSA-1889981

Type

Reaction [transition]

Species

Homo sapiens

Compartment

Golgi membrane, Golgi lumen

Synonyms

Galactose is the second sugar added in the tetrasaccharide linker

ReviewStatus

5/5

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B4GALT7 transfers Gal group to xylosyl-unit of the tetrasaccharide linker

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Beta-1,4-galactosyltransferase 7 (B4GALT7) adds galactose (Gal) to beta-xyloside in a beta-1,4 linkage creating the second unit in the formation of the tetrasaccharide linker, the precursor required for glycosaminoglycan (GAG) synthesis (Almeida et al. 1999). Defects in B4GALT7 cause Ehlers-Danlos syndrome progeroid type (EDSP) (MIM:130070) (Okajima et al. 1999).

Literature References

PubMed ID	Title	Journal	Year
10506123	Molecular basis for the progeroid variant of Ehlers-Danlos syndrome. Identification and characterization of two mutations in galactosyltransferase I gene Fukumoto, S, Urano, T, Okajima, T, Furukawa, K	J Biol Chem	1999
10473568	Cloning and expression of a proteoglycan UDP-galactose:beta-xylose beta1,4-galactosyltransferase I. A seventh member of the human beta4-galactosyltransferase gene family Mandel, U, Almeida, R, Bennett, EP, Schwientek, T, Levery, SB, Clausen, H, Kresse, H	J Biol Chem	1999