The present invention relates to substantially pure mannan-binding lectin
associated serine protease-2 (MASP-2) polypeptides and fragments thereof
as well as nucleic acids encoding such polpeptides. Furthermore, the
present invention relates to uses of a substantially pure polypeptide
comprising amino acid sequences derived from mannan-binding lectin
associated serine protease-2 (MASP2) or a functional homologue thereof
for the production of a pharmaceutical composition as well as
pharmaceutical compositions comprising MASP-2 and/or MASP-2 fragments. In
addition the present invention relates to inhibitors of MASP-2 and
pharmaceutical compositions comparing such inhibitors. Methods for
detecting MASP-2 nucleic acid expression are included in the invention.