Frequency of COL4A3/COL4A4 mutations amongst families segregating glomerular microscopic hematuria and evidence for activation of the unfolded protein response. Focal and segmental glomerulosclerosis is a frequent development during ageing.

Cells expressing the mutant chains showed a trend towards higher accumulation within the cell as revealed by western blotting of the cellular lysate 48-hrs after transfection ( Fig. 2 ).

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The results demonstrated a clear trend towards increased cellular retaining of the mutant chains as compared to the WT ( Fig. 2 ), a finding consistent with previous results [19] , [32] .

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