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Patients carrying at least one missense variation, with a positive canalicular expression of MDR3 and a biliary phospholipid level over 6.9% of total biliary lipid levels, presented a better response to UDCA and more prolonged native liver survival [91]

When the motor neurons die, the ability of the brain to initiate and control muscle movement is lost

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These include a degree of weight loss (thought to be due to glucose excretion and fluid depletion), but also a reduction in blood pressure as a result of volume depletion, sodium excretion, weight loss and reduction in reninangiotensinaldosterone system (RAAS) activity [151, 152]