KLHL3 and metabolic acidosis: Knock-in mice carrying KLHL3 disease causing mutations exhibited salt-sensitive hypertension, hyperkalaemia, and metabolic acidosis and revealed that both WNK1 and WNK4 were increased within the kidney due to impaired KLHL3-Cullin 3-mediated ubiquitination [104] Homozygous knockout KLHL3 mice models also produced a Gordon syndrome phenotype with increased WNK1 and WNK4 levels in the kidney [105].