This is a family conserved from bacteria to humans. The structure of a member from Bacteroides has been crystallised and modelled onto the luminal region of the human member of the family, the transmembrane glycoprotein N-acetylglucosamine-1-phosphodiester alpha-N-acetylglucosaminidase. There is some conservation of potentially functional residues, implying that in the bacterial members this family acts in some way as a phosphodiester glycosidase [1]. The human protein is also present, so the eukaryotic members are likely to be catalysing the second step in the formation of the mannose 6-phosphate targeting signal on lysosomal enzyme oligosaccharides [2]. [1]. 23572527. Structure and function of the DUF2233 domain in bacteria and in the human mannose 6-phosphate uncovering enzyme. Das D, Lee WS, Grant JC, Chiu HJ, Farr CL, Vance J, Klock HE, Knuth MW, Miller MD, Elsliger MA, Deacon AM, Godzik A, Lesley SA, Kornfeld S, Wilson IA;. J Biol Chem. 2013;288:16789-16799. [2]. 20147709. Mutations in the lysosomal enzyme-targeting pathway and persistent stuttering. Kang C, Riazuddin S, Mundorff J, Krasnewich D, Friedman P, Mullikin JC, Drayna D;. N Engl J Med. 2010;362:677-685. (from Pfam)
- Date:
- 2024-10-16