ALG8 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 the lipid-linked oligosaccharide Glc(1)Man(9)GlcNAc(2)-PP-Dol before it is transferred to the nascent peptide. Required for PKD1/Polycystin-1 maturation and localization to the plasma membrane of the primary cilia. Belongs to the ALG6/ALG8 glucosyltransferase family. 2 alternatively spliced human isoforms have been reported. Note: This description may include information from UniProtKB.
Protein type: EC; Glycan Metabolism - N-glycan biosynthesis; Membrane protein, integral; Membrane protein, multi-pass; Transferase
Chromosomal Location of Human Ortholog: 11q14.1
Cellular Component:  endoplasmic reticulum membrane; integral component of membrane
Molecular Function:  alpha-1,3-mannosyltransferase activity; dolichyl pyrophosphate Glc1Man9GlcNAc2 alpha-1,3-glucosyltransferase activity; dolichyl-phosphate-glucose-glycolipid alpha-glucosyltransferase activity; protein binding
Biological Process:  dolichol-linked oligosaccharide biosynthetic process; mannosylation; protein N-linked glycosylation; protein N-linked glycosylation via asparagine
Disease: Congenital Disorder Of Glycosylation, Type Ih; Polycystic Liver Disease 3 With Or Without Kidney Cysts
Reference #:  Q9BVK2 (UniProtKB)
Alt. Names/Synonyms: ALG8; ALG8 alpha-1,3-glucosyltransferase; asparagine-linked glycosylation 8 alpha-13-glucosyltransferase-like protein; asparagine-linked glycosylation 8 homolog (S. cerevisiae, alpha-1,3-glucosyltransferase); asparagine-linked glycosylation 8 homolog (yeast, alpha-1,3-glucosyltransferase); asparagine-linked glycosylation 8, alpha-1,3-glucosyltransferase homolog; asparagine-linked glycosylation 8, alpha-1,3-glucosyltransferase homolog (S. cerevisiae); Asparagine-linked glycosylation protein 8 homolog; CDG1H; Dol-P-Glc:Glc(1)Man(9)GlcNAc(2)-PP-dolichyl alpha-1,3-glucosyltransferase; dolichyl pyrophosphate Glc1Man9GlcNAc2 alpha-1,3-glucosyltransferase; dolichyl-P-Glc:Glc(1)Man(9)GlcNAc(2)-PP-dolichol alpha- 1->3-glucosyltransferase; Dolichyl-P-Glc:Glc1Man9GlcNAc2-PP-dolichyl glucosyltransferase; dolichyl-P-glucose:Glc1Man9GlcNAc2-PP-dolichyl-alpha-1,3-glucosyltransferase; HUSSY-02; MGC2840; PCLD3; Probable dolichyl pyrophosphate Glc1Man9GlcNAc2 alpha-1,3-glucosyltransferase
Gene Symbols: ALG8
Molecular weight: 60,088 Da
Basal Isoelectric point: 9.28  Predict pI for various phosphorylation states
Select Structure to View Below


Protein Structure Not Found.

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