dolichyl pyrophosphate Glc1man9GlcNAc2 alpha-1,3-glucosyltransferase; asparagine-linked glycosylation protein 8 homolog; dol-P-Glc:Glc(1)man(9)GlcNAc(2)-PP-dolichyl alpha-1,3-glucosyltransferase; dolichyl-P-Glc:Glc1man9GlcNAc2-PP-dolichyl glucosyltransferase (ALG8, HUSSY-2)

Function

• adds the second glucose residue to the lipid-linked oligosaccharide precursor for N-linked glycosylation
• transfers glucose from dolichyl phosphate glucose (dol-P-Glc) onto thelipid-linked oligosaccharide Glc(1)man(9)GlcNAc(2)-PP-dol
• protein modification; protein glycosylation

Structure

• belongs to the ALG6/ALG8 glucosyltransferase family

Compartment

• endoplasmic reticulum membrane
• multi-pass membrane protein (putative)

Pathology

• defects in ALG8 are the cause of congenital disorder of glycosylation type 1H

More general terms

• glucosyl transferase
• transmembrane 11 protein

Additional terms

• congenital disorder of glycosylation type 1H
• HUSSY protein

Database

• Entrez gene: http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&cmd=Retrieve&dopt=Graphics&list_uids=79053
• Kegg: http://www.genome.jp/dbget-bin/www_bget?hsa:79053
• OMIM: https://mirror.omim.org/entry/608103
• OMIM: https://mirror.omim.org/entry/608104
• UniProt: http://www.uniprot.org/uniprot/Q9BVK2.html