Schematic representation of cochlin domain structure with an N-terminal signal peptide
(SP), followed by a Limulus factor C, cochlin, and late gestation lung protein, Lgl1
(LCCL) domain, two von Willebrand factor A-like (vWFA) domains, and two short lengths of
intervening domains (ivd1 and ivd2). A total of 21 mutations have been reported, eight of
which (marked red) were investigated in this study. Three HA epitope tags
were added at the C-terminal end of the COCH sequences (C=cysteine
residue).