alpha-1,2-glucosyltransferase ALG10-B (alpha-2-glucosyltransferase ALG10-B, asparagine-linked glycosylation protein 10 homolog B, K+ channel regulator 1, K+ channel, voltage-gated subfamily H member 2, KCNH2, human ether-a-go-go-related gene protein, HERG, ERG1 ALG10B, KCR1)

Function

• glycosylation pathway
• alpha-1,2-glucosyltransferase (putative_
• adds 3rd glucose residue to the lipid-linked oligosaccharide precursor for N-linked glycosylation
• transfers glucose from dolichyl phosphate glucose (Dol-P-Glc) onto lipid-linked oligosaccharide Glc(2)Man(9)GlcNAc(2)-PP-Dol
• when coupled to KCNH2 may reduce KCNH2 sensitivity to classic proarrhythmic drug blockade, possibly by mediating glycosylation of KCNH2
• interacts with KCNH1 & KCNH2/HERG

Compartment

• cell membrane

Expression

• expressed in heart, placenta, liver, kidney, pancreas > lung, skeletal muscle, brain.

Pathology

• defects in ALG10B may diminish susceptibility to acquired long QT syndrome

More general terms

• glucosyl transferase
• transmembrane 12 protein

Database

• OMIM: https://mirror.omim.org/entry/152427
• OMIM: https://mirror.omim.org/entry/603313
• UniProt: http://www.uniprot.org/uniprot/Q5I7T1.html