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Cholestasis: CANALICULAR SECRETION Part 4

ATP-independent transport The canalicular membrane also contains transport processes that are not energy dependent, and thus do not require ATP. For example, the chloride/bicarbonate anion exchanger...

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Cholestasis: MECHANISMS OF CHOLESTASIS – DEFECTS IN TRANSPORT PROTEINS Part 1

Transport function is dynamically altered when cholestasis arises. Different transporters are variably altered. Some are downregulated to cause or contribute to the cholestasis, whereas others are...

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Cholestasis: MECHANISMS OF CHOLESTASIS – DEFECTS IN TRANSPORT PROTEINS Part 2

YGT level suggests that bile salt secretion is so severely impaired that it does not reach the canaliculi and, therefore, does not invoke canalicular or cholangiolar damage. Histology confirms the...

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Cholestasis: MECHANISMS OF CHOLESTASIS – DEFECTS IN TRANSPORT PROTEINS Part 3

Many different mutations of the MDR3 gene (on chromosome 7q21) can occur, leading to a variety of clinical manifestations. Mutations that completely abrogate MDR3 function result in the onset of severe...

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Cholestasis: MECHANISMS OF CHOLESTASIS – DEFECTS IN TRANSPORT PROTEINS Part 4

Acquired cholestatic syndromes In cholestatic illnesses, most changes in transporter expression in hepatocytes and cholangiocytes are actually adaptive responses that limit the accumulation of...

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Cholestasis: MECHANISMS OF CHOLESTASIS – DEFECTS IN TRANSPORT PROTEINS Part 5

Basolateral membrane transport protein changes: Many of the changes that occur in this domain are reactive in nature and help to protect the hepatocyte from the effects of potentially toxic compounds:...

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Cholestasis: MECHANISMS OF CHOLESTASIS – DEFECTS IN TRANSPORT PROTEINS Part 6

Changes in cholangiocyte transport: Although cholangio-cytes are the primary target for the cholestasis in the various ‘vanishing bile duct syndromes’, including PBC, limited information is available...

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Cholestasis: INTERPRETATION OF THE CASE

The phenotypic features of the child described in the ‘Case Presentation’ — early onset of cholestasis, high serum yGT, and bile duct injury with portal inflammation that progressed to cirrhosis and...

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Cholestasis: SUMMARY Part 1

The hepatocyte is a polarized epithelial cell with two distinctive domains: basolateral (sinusoidal) and canalicular (apical). Transport proteins located in the basolateral and canalicular membranes...

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Cholestasis: SUMMARY Part 2

Genetic defects in these canalicular transporters are responsible for several types of PFIC. PFIC-1 results from a mutation of the FIC1 gene. It also exhibits phenotypic heterogeneity: less severe...

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