B4GALT5

This gene is one of seven beta-1,4-galactosyltransferase (beta4GalT) genes. They encode type II membrane-bound glycoproteins that appear to have exclusive specificity for the donor substrate UDP-galactose; all transfer galactose in a beta1,4 linkage to similar acceptor sugars: GlcNAc, Glc, and Xyl. Each beta4GalT has a distinct function in the biosynthesis of different glycoconjugates and saccharide structures. As type II membrane proteins, they have an N-terminal hydrophobic signal sequence that directs the protein to the Golgi apparatus and which then remains uncleaved to function as a transmembrane anchor. By sequence similarity, the beta4GalTs form four groups: beta4GalT1 and beta4GalT2, beta4GalT3 and beta4GalT4, beta4GalT5 and beta4GalT6, and beta4GalT7. The function of the enzyme encoded by this gene is not clear. This gene was previously designated as B4GALT4 but was renamed to B4GALT5. In the literature it is also referred to as beta4GalT2. [provided by RefSeq, Jul 2008]

Full Name

Beta-1,4-Galactosyltransferase 5

Function

Catalyzes the synthesis of lactosylceramide (LacCer) via the transfer of galactose from UDP-galactose to glucosylceramide (GlcCer) (PubMed:24498430).
LacCer is the starting point in the biosynthesis of all gangliosides (membrane-bound glycosphingolipids) which play pivotal roles in the CNS including neuronal maturation and axonal and myelin formation (By similarity).
Plays a role in the glycosylation of BMPR1A and regulation of its protein stability (By similarity).
Essential for extraembryonic development during early embryogenesis (By similarity).

Biological Process

Central nervous system myelination Source: UniProtKB
Central nervous system neuron axonogenesis Source: UniProtKB
Ganglioside biosynthetic process via lactosylceramide Source: UniProtKB
Glycoprotein biosynthetic process Source: GO_Central
Glycosylation Source: GO_Central
Keratan sulfate biosynthetic process Source: Reactome
Neuron maturation Source: UniProtKB
O-glycan processing Source: Reactome
Poly-N-acetyllactosamine biosynthetic process Source: Ensembl
Positive regulation of embryonic development Source: UniProtKB
Protein glycosylation Source: UniProtKB
Regulation of protein stability Source: UniProtKB

Cellular Location

Golgi stack membrane; Golgi apparatus. Trans cisternae of Golgi stack.

Topology

Cytoplasmic: 1-14 aa
Helical: 15-35 aa
Lumenal: 36-388 aa