Mutations in vacuolar H+-ATPase subunits lead to biliary developmental defects in zebrafish

► We report the identification of three novel zebrafish biliary mutants. ► Pekin is caused by mutation in the vacuolar H+-ATPase subunit gene atp6ap2. ► Pekin also demonstrates hypopigmentation and defects in pancreas and intestine. ► Other V-H+-ATPase mutants have similar defects. ► Inhibition of atp6ap2 and the trafficking gene vps33b is additive.