Biochemical mechanisms of aggregation in TGFBI-linked corneal dystrophies Nadia Sukusu Nielsena, Ebbe Toftgaard Poulsena, Marie V. Lukassenb, Connie Chao Shernc,d, Emilie Hage Mogensena, Christian E. Weberskova, Larry DeDionisiod, Leif Schausere, Tara C. B. Moorec,d, Daniel E. Otzena,f, Jesper Hjortdalg, Jan J. Enghilda,* a Department of Molecular Biology and Genetics, Aarhus University, Aarhus, Denmark b Biomolecular Mass Spectrometry and Proteomics, Utrecht Institute for Pharmaceutical Sciences, University of Utrecht, Utrecht, the Netherlands c Biomedical Sciences Research Institute, Ulster University, Coleraine, Northern Ireland, UK d Avellino Labs USA, Menlo Park, USA e QIAGEN Aarhus A/S, Aarhus, Denmark f Interdisciplinary Nanoscience Centre (iNANO), Aarhus University, Aarhus, Denmark g Department of Ophthalmology, Aarhus University Hospital, Aarhus, Denmark * Corresponding author. Department of Molecular Biology and Genetics, Aarhus University, Gustav Wieds vej 10C, Aarhus, Denmark. E-mail:
[email protected] Author contributions (%) The percentage of work contributed by each author in the production of the manuscript is as follows: NSN 25%, ETP 10%, MVL 10%, CCS 5%, EHM 2%, CEW 2%, LD 2%, LS 4%, TCBM 5%, DEO 10%, JH 10%, and JJE 15%. 1 ABSTRACT Transforming growth factor-β-induced protein (TGFBIp), an extracellular matrix protein, is the second most abundant protein in the corneal stroma. In this review, we summarize the current knowledge concerning the expression, molecular structure, binding partners, and functions of human TGFBIp.