ER degradation-enhancing alpha-mannosidase-like protein 3

Definition:

Involved in endoplasmic reticulum-associated degradation (ERAD). Accelerates the glycoprotein ERAD by proteasomes, by catalyzing mannose trimming from Man8GlcNAc2 to Man7GlcNAc2 in the N-glycans. May also participate in mannose trimming from all glycoproteins and not just misfolded ones targeted to ERAD. May have alpha 1,2-mannosidase activity (By similarity).

References:

[1]. Satoshi Ninagawa, et al. EDEM2 initiates mammalian glycoprotein ERAD by catalyzing the first mannose trimming step. J Cell Biol. 2014 Aug 4;206(3):347-56. [Content Brief]

[2]. Daniel L Polla, et al. Bi-allelic variants in the ER quality-control mannosidase gene EDEM3 cause a congenital disorder of glycosylation. Am J Hum Genet. 2021 Jul 1;108(7):1342-1349. [Content Brief]

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