A third mannose is added to the N-glycan precursor by ALG2 using its alpha1,6-mannosyltransferase activity. This has been demonstrated experimentally in yeast (O'Reilly MK et al, 2006; Kämpf M et al, 2009); the human reaction is inferred by homology. Defects in ALG2 are the cause for CDG1I (Thiel C et al, 2003).
Lehle, L, Absmanner, B, Kämpf, M, Schwarz, M
Zhang, G, O'Reilly, MK, Imperiali, B
Braulke, T, Körner, C, Lehle, L, Grzmil, M, Peng, J, Kohlschütter, A, von Figura, K, Thiel, C, Hasilik, M, Schwarz, M
alpha-1,6-mannosyltransferase activity of ALG2 [endoplasmic reticulum membrane]
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