Poster Presentation 50th Lorne Proteins Conference 2025

Expanding substrate diversity of O-fucosylation through structural alignment and glycoproteomics (#143)

Benjamin Eberand 1 , Michelle Cielesh 1 , Kiran Muthukrishnan 1 , Huilin Hao 2 , Freda Passam 1 , Robert Haltiwanger 2 , Mark Larance 1
  1. University of Sydney, Camperdown, NSW, Australia
  2. Complex Carbohydrate Research Center, University of Georgia, Athens, Georgia, USA

Protein O-fucosyltransferase (POFUT) enzymes mediate direct O-fucosylation on secreted and membrane-associated proteins such as thrombospondin-1 and Notch-11,2. Dysregulation of O-fucosylation has been linked to numerous cancers and developmental disorders3. POFUTs play a key role in modulating the secretion of their substrates, by O-fucosylating specific substrate protein domains only when correctly folded, and as such are thought to be involved in non-canonical protein quality control4. However, as O-glycosylation is critically understudied, the true diversity of proteins regulated by O-fucosylation is largely uncharacterised5.

We recently identified the human FUT10/FUT11 enzymes as responsible for protein O-fucosylation of Elastin Microfibril Interface (EMI) domains6. Here, we combined Alphafold2 protein structural prediction and the structural alignment tool Foldseek to identify new substrates for FUT10/FUT11. Each human EMI-domain was analysed in Foldseek using an iterative approach to detect structurally homologous protein domains. Numerous proteins with EMI-like domains were identified, including microfibrillar-associated protein 2/5 (MFAP2/MFAP5), which have key roles in signalling within the extracellular matrix. The structure of the EMI-like domain is highly conserved in these proteins despite low primary sequence homology. As such, we hypothesised they may be recognised by the structurally-specific FUT10/FUT11 enzymes. O-fucosylation within these new EMI-like domains was confirmed at high stoichiometry in mouse tissues. To identify the enzyme modifying these proteins, HEK293 cells were transiently transfected with MFAP2/5 with or without FUT10/FUT11 to quantify interactions and O-fucosylation. Interestingly, O-fucosylation of MFAP2/MFAP5 is only observed in cells co-transfected with FUT10, but not with FUT11, a selectivity that has not been seen for EMI domains. To functionally characterise O-fucosylation of these EMI-like domains, we performed secretion assays comparing wild-type MFAP2 with an alanine mutant at the modified residue, preventing O-fucosylation. This mutant showed >50% loss in secretion, indicating that FUT10 is required for proper secretion of MFAP2. Future work will investigate the role of this O-fucosylation through FUT10/11 knockout mice, and streamline this workflow for application to other enzymes. A methodology for rapid identification of new enzyme substrates would allow a deeper understanding of the diverse roles enzymes may play in different contexts and broaden our knowledge of the human interactome.

  1. Du, J. G. et al. O-fucosylation of thrombospondin type 1 repeats restricts epithelial to mesenchymal transition (EMT) and maintains epiblast pluripotency during mouse gastrulation. Developmental Biology 346, 25-38 (2010). https://doi.org:10.1016/j.ydbio.2010.07.008
  2. Pennarubia, F., Ito, A., Takeuchi, M. & Haltiwanger, R. S. Cancer-associated Notch receptor variants lead to O-fucosylation defects that deregulate Notch signaling. Journal of Biological Chemistry 298 (2022). https://doi.org:10.1016/j.jbc.2022.102616
  3. Weng, A. P. et al. Activating mutations of NOTCH-1 in human T cell acute lymphoblastic leukemia. Science 306, 269-271 (2004). https://doi.org:10.1126/science.1102160
  4. Holdener, B. C. & Haltiwanger, R. S. Protein O-fucosylation: structure and function. Curr Opin Struct Biol 56, 78-86 (2019). https://doi.org:10.1016/j.sbi.2018.12.005
  5. Thompson, N. & Wakarchuk, W. O-glycosylation and its role in therapeutic proteins. Biosci Rep 42 (2022). https://doi.org:10.1042/BSR20220094
  6. Houlahan, C. B. et al. Analysis of the Healthy Platelet Proteome Identifies a New Form of Domain-Specific O-Fucosylation. Mol Cell Proteomics 23, 100717 (2024). https://doi.org:10.1016/j.mcpro.2024.100717