Alpha-1,2-glucosyltransferase ALG10-A (ALG10)

Alpha-1,2-glucosyltransferase ALG10-A (ALG10) is a membrane-bound glycosyltransferase residing in the endoplasmic reticulum that catalyzes the final step of N-linked glycosylation by transferring the third and last glucose residue from dolichyl phosphate glucose (Dol-P-Glc) to the lipid-linked oligosaccharide intermediate Glc(2)Man(9)GlcNAc(2)-PP-Dol, producing Glc(3)Man(9)GlcNAc(2)-PP-Dol. This residue is required for proper recognition by quality-control chaperones like calnexin and calreticulin, supporting protein folding and maturation. Defects in ALG10 result in congenital disorders of glycosylation, characterized by improper glycoprotein folding and widespread systemic effects including neurologic, muscular, and metabolic dysfunction. The gene is also known to have regulatory effects on potassium ion channels, which may influence cardiac rhythm stability and neurological processes.

Other names

ALG10ALG10ADIE2Dolichyl-P-Glc:Glc(2)Man(9)GlcNAc(2)-PP-Dol alpha-1,2-glucosyltransferaseDol-P-Glc:Glc(2)Man(9)GlcNAc(2)-PP-Dol alpha-1,2-glucosyltransferaseAsparagine-linked glycosylation protein 10 homolog AAlpha-1,2-glucosyltransferase ALG10-AAlpha-2-glucosyltransferase ALG10-AFLJ14751Asparagine-linked glycosylation 10, alpha-1,2-glucosyltransferase homolog (S. pombe/yeast)Derepression of ITR1 expression 2 homologPotassium channel regulator 1 (KCR1)EC 2.4.1.256EC 2.4.1.16EC 2.4.1

Not applicable; no drugs are known to directly target ALG10. Potential role in modifying ion channel pharmacology indirectly, impacting drug sensitivity (e.g., HERG channel block).