Alpha-1,2-glucosyltransferase ALG10B (ALG10B)

Alpha-1,2-glucosyltransferase ALG10B (ALG10B) is an endoplasmic reticulum membrane-associated enzyme that catalyzes the terminal addition of glucose to lipid-linked oligosaccharide precursors during the process of N-linked glycosylation in eukaryotic cells[1][2][3]. This last glucose addition is essential for proper folding and quality control of nascent glycoproteins through the calnexin/calreticulin cycle. Failure of this process impacts protein homeostasis and can result in disease, with mutations in ALG10B causing nonsyndromic hearing impairment due to cochlear outer hair cell dysfunction, and deletions or dysfunction contributing to cardiac electrical disturbances such as long QT syndrome type 2[1][2][3]. ALG10B is also known to influence the glycosylation state of potassium channels (e.g., HERG/KCNH2), potentially modulating their sensitivity to certain drugs, making this enzyme relevant for understanding channelopathies and posttranslational regulation of ion channels[1][2][3].

Other names

ALG10KCR1Potassium channel regulator 1Dolichyl-P-Glc:Glc(2)Man(9)GlcNAc(2)-PP-Dolichol alpha-1,2-glucosyltransferaseDol-P-Glc:Glc(2)Man(9)GlcNAc(2)-PP-Dol alpha-1,2-glucosyltransferaseAsparagine-linked glycosylation protein 10 homolog BAlpha-1,2-glucosyltransferase ALG10-AAlpha-2-glucosyltransferase ALG10-BModifier of the HERG potassium channelEC 2.4.1.256EC 2.4.1.16

Modulation of glycosylation may affect drug sensitivity of potassium channels (e.g., HERG channel via glycosylation-dependent changes)