We propose that in dRTA patients where kAE1 function is abnormal due to a mutation in the SLC4A1 gene, the functional interaction between kAE1 and claudin-4 is altered, thus resulting in a tighter collecting duct epithelium preventing sodium and chloride reabsorption45 and thus loss of urinary ions. This evidence concerns the gene SLC4A1 and distal renal tubular acidosis.