Amongst the 70 or so ENaC variants known to cause PHA-1B, 41 occur in SCNN1A, encoding the α-subunit, 21 in SCNN1B, encoding the β-subunit and 7 in SCNN1G, encoding the γ-subunit (Table 1). The gene discussed is SCNN1A; the disease is pseudohypoaldosteronism, type IB1, autosomal recessive.