▪ Abstract The primary or hereditary form of distal renal tubular acidosis (dRTA), although
rare, has received increased attention recently because of dramatic advances in the
understanding of its genetic basis. The final regulation of renal acid excretion is effected by
various acid/base transporters localized in specialized cells in the cortical collecting and
outer medullary collecting tubules. Inherited defects in two of the key acid/base transporters
involved in distal acidification, as well as mutations in the cytosolic carbonic anhydrase …