The Na-K-Cl co-transporter NKCC1 uses the Na+ gradient to accumulate chloride into the cell. It is broadly expressed and involved in different cellular processes including transepithelial ion transport, cell volume regulation, and intracellular chloride homeostasis. Consequently, disruption of NKCC1 results in a complex phenotype with deafness, circling behavior, male infertility, and impaired salivation.