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A proposed model for ATP8B1 function at the bile canalicular membrane. Deficiency of ATP8B1 leads to loss of maintenance of aminophospholipids on the cytoplasmic side of the canalicular membrane bilayer making the canalicular membrane susceptible to disruption by hydrophobic bile acids, such as CDCA, a primary human bile acid. In contrast, the hydrophilic bile acid, UDCA does not have the same disruptive effect.
