[HTML][HTML] The Brichos domain-containing C-terminal part of pro-surfactant protein C binds to an unfolded poly-val transmembrane segment

H Johansson, K Nordling, TE Weaver… - Journal of biological …, 2006 - ASBMB
Native lung surfactant protein C (SP-C) is a 4.2-kDa acylpeptide that associates with
alveolar surfactant phospholipids via a transmembrane α-helix. This helix contains mainly
Val, although poly-Val is inefficient in helix formation, and helical SP-C can spontaneously
convert to β-sheet aggregates and amyloid-like fibrils. SP-C is cleaved out from a 21-kDa
integral membrane protein, proSP-C, in the alveolar type II cell. Recently several mutations
localized in the endoplasmic reticulum-lumenal (C-terminal) part of proSP-C (CTproSP-C) …