Abstract: A novel gene defining a novel enzyme UDP-Galactose: &bgr;-D-Galactose-R 4-&agr;-D-galactosyltransferase, termed &agr;4Gal-T1, with unique enzymatic properties is disclosed. The invention discloses isolated DNA molecules and DNA constructs encoding &agr;4Gal-T1 and derivatives thereof by way of amino acid deletion, substitution or insertion exhibiting &agr;4Gal-T1 activity, as well as cloning and expression vectors including such DNA, cells transfected with vectors, and recombinant methods for providing &agr;4Gal-T1. The enzyme &agr;4Gal-T1 and &agr;4Gal-active derivatives thereof are disclosed. Further, the invention discloses methods of obtaining &agr;1, 4galactosyl glycosylated glycosphingolipids by use of an enzymatically active &agr;4Gal-T1 protein thereof or by using cells stably transfected with a vector including DNA encoding an enzymatically active &agr;4Gal-T1 protein as an experession system for recombinant production of such glycosphingolipids.