Abstract:
:Fibulin-4 is a matricellular protein required for extracellular matrix (ECM) assembly. Mice deficient in fibulin-4 (Fbln4-/- ) have disrupted collagen and elastin fibers and die shortly after birth from aortic and diaphragmatic rupture. The function of fibulin-4 in ECM assembly, however, remains elusive. Here, we show that fibulin-4 is required for the activity of lysyl oxidase (LOX), a copper-containing enzyme that catalyzes the covalent cross-linking of elastin and collagen. LOX produced by Fbln4-/- cells had lower activity than LOX produced by wild-type cells due to the absence of lysine tyrosyl quinone (LTQ), a unique cofactor required for LOX activity. Our studies showed that fibulin-4 is required for copper ion transfer from the copper transporter ATP7A to LOX in the trans-Golgi network (TGN), which is a necessary step for LTQ formation. These results uncover a pivotal role for fibulin-4 in the activation of LOX and, hence, in ECM assembly.
journal_name
Sci Advjournal_title
Science advancesauthors
Noda K,Kitagawa K,Miki T,Horiguchi M,Akama TO,Taniguchi T,Taniguchi H,Takahashi K,Ogra Y,Mecham RP,Terajima M,Yamauchi M,Nakamura Tdoi
10.1126/sciadv.abc1404subject
Has Abstractpub_date
2020-11-25 00:00:00issue
48issn
2375-2548pii
6/48/eabc1404journal_volume
6pub_type
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