Fig. 2.
Laminin containing an S68R amino acid substitution in the β-LN domain does not polymerize and does not assemble a basement membrane. A-C. β2-S80 is a conserved residue and patients with a laminin β2-S80R mutation develop Pierson syndrome. The ability of laminin-111 bearing the corresponding mutation in β1LN to polymerize was evaluated in a polymerization sedimentation assay. Effectively, no polymer was detected in the indicated concentration range. D–E. Laminin assembly was evaluated on Schwann cell surfaces at 28 nM laminin alone or in the presence of 28 nM nidogen-1 and 14 nM collagen. WT laminin accumulated substantially while Lmβ1 S68R did not.