Importantly, mice with a disruption on Atp6v1b1, the ortholog of which in humans causes dRTA, also lack the hypercalciuria, nephrocalcinosis and overt acidemia (Gueutin et al., 2013) seen in these patients, pointing to some limitations inherent to these mouse models. This evidence concerns the gene ATP6V1B1 and distal renal tubular acidosis.