Figure 4

Figure 4

Reduced levels and defective copper induced trafficking of the MNK protein in primary fibroblasts with the MNK/III.6 mutation. (A) Immunoblot detection of MNK protein levels in membrane preparations from the primary fibroblast cell lines. The GM13672 cell line was derived from Menkes patient 1433, an affected cousin of patient III.6 with the same mutation, and the GM00302 and GM00970 cells are normal primary fibroblasts. (B-G) Immunofluorescence microscopy with affinity purified MNK antibodies to detect the location of MNK protein in the cultured primary cells exposed to basal media or for two hours in media containing 200 μmol/l copper (+Cu).