Matches in SemOpenAlex for { <https://semopenalex.org/work/W2023471754> ?p ?o ?g. }
- W2023471754 endingPage "204" @default.
- W2023471754 startingPage "195" @default.
- W2023471754 abstract "Progressive familial intrahepatic cholestasis type 1 (PFIC1, Byler disease, OMIM 211600) is a severe inherited liver disease caused by mutations in ATP8B1. ATP8B1 is a member of the type 4 subfamily of P-type ATPases, which are phospholipid flippases. PFIC1 patients generally develop end-stage liver disease before the second decade of life. The disease is characterized by impaired biliary bile salt excretion, but the mechanism whereby impaired ATP8B1 function results in cholestasis is unclear. In a mouse model for PFIC1, we observed decreased resistance of the hepatocanalicular membrane to hydrophobic bile salts as evidenced by enhanced biliary recovery of phosphatidylserine, cholesterol, and ectoenzymes. In liver specimens from PFIC1 patients, but not in those from control subjects, ectoenzyme expression at the canalicular membrane was markedly deficient. In isolated mouse livers Atp8b1 deficiency impaired the transport of hydrophobic bile salts into bile. In conclusion, our study shows that Atp8b1 deficiency causes loss of canalicular phospholipid membrane asymmetry that in turn renders the canalicular membrane less resistant toward hydrophobic bile salts. The loss of phospholipid asymmetry may subsequently impair bile salt transport and cause cholestasis. (HEPATOLOGY 2006;44:195–204.)" @default.
- W2023471754 created "2016-06-24" @default.
- W2023471754 creator A5000234813 @default.
- W2023471754 creator A5014820840 @default.
- W2023471754 creator A5020023657 @default.
- W2023471754 creator A5023577779 @default.
- W2023471754 creator A5024375351 @default.
- W2023471754 creator A5036909379 @default.
- W2023471754 creator A5038978687 @default.
- W2023471754 creator A5040740846 @default.
- W2023471754 creator A5044314042 @default.
- W2023471754 creator A5044963611 @default.
- W2023471754 creator A5047244385 @default.
- W2023471754 creator A5057283287 @default.
- W2023471754 creator A5064600273 @default.
- W2023471754 creator A5087413894 @default.
- W2023471754 creator A5090844389 @default.
- W2023471754 date "2006-07-01" @default.
- W2023471754 modified "2023-10-06" @default.
- W2023471754 title "Atp8b1 deficiency in mice reduces resistance of the canalicular membrane to hydrophobic bile salts and impairs bile salt transport" @default.
- W2023471754 cites W1612407641 @default.
- W2023471754 cites W1751757376 @default.
- W2023471754 cites W1943976550 @default.
- W2023471754 cites W1973679514 @default.
- W2023471754 cites W1980151643 @default.
- W2023471754 cites W1982558653 @default.
- W2023471754 cites W1987247771 @default.
- W2023471754 cites W1989126866 @default.
- W2023471754 cites W1990637933 @default.
- W2023471754 cites W2005825651 @default.
- W2023471754 cites W2007795329 @default.
- W2023471754 cites W2007879482 @default.
- W2023471754 cites W2009084021 @default.
- W2023471754 cites W2014300627 @default.
- W2023471754 cites W2017074521 @default.
- W2023471754 cites W2017799662 @default.
- W2023471754 cites W2019793098 @default.
- W2023471754 cites W2022903648 @default.
- W2023471754 cites W2025406853 @default.
- W2023471754 cites W2033265977 @default.
- W2023471754 cites W2041376402 @default.
- W2023471754 cites W2046338633 @default.
- W2023471754 cites W2050007442 @default.
- W2023471754 cites W2051441289 @default.
- W2023471754 cites W2055306045 @default.
- W2023471754 cites W2061569374 @default.
- W2023471754 cites W2063720581 @default.
- W2023471754 cites W2069568296 @default.
- W2023471754 cites W2069612904 @default.
- W2023471754 cites W2078844117 @default.
- W2023471754 cites W2096598361 @default.
- W2023471754 cites W2101690661 @default.
- W2023471754 cites W2108293406 @default.
- W2023471754 cites W2121537182 @default.
- W2023471754 cites W2135585405 @default.
- W2023471754 cites W2138952800 @default.
- W2023471754 cites W2142083551 @default.
- W2023471754 cites W2142658372 @default.
- W2023471754 cites W2145736004 @default.
- W2023471754 cites W2156504663 @default.
- W2023471754 cites W2163809251 @default.
- W2023471754 cites W2168526937 @default.
- W2023471754 cites W2184070211 @default.
- W2023471754 cites W2319534865 @default.
- W2023471754 cites W2328987984 @default.
- W2023471754 cites W2338803779 @default.
- W2023471754 cites W2340109465 @default.
- W2023471754 cites W2996497172 @default.
- W2023471754 doi "https://doi.org/10.1002/hep.21212" @default.
- W2023471754 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/16799980" @default.
- W2023471754 hasPublicationYear "2006" @default.
- W2023471754 type Work @default.
- W2023471754 sameAs 2023471754 @default.
- W2023471754 citedByCount "211" @default.
- W2023471754 countsByYear W20234717542012 @default.
- W2023471754 countsByYear W20234717542013 @default.
- W2023471754 countsByYear W20234717542014 @default.
- W2023471754 countsByYear W20234717542015 @default.
- W2023471754 countsByYear W20234717542016 @default.
- W2023471754 countsByYear W20234717542017 @default.
- W2023471754 countsByYear W20234717542018 @default.
- W2023471754 countsByYear W20234717542019 @default.
- W2023471754 countsByYear W20234717542020 @default.
- W2023471754 countsByYear W20234717542021 @default.
- W2023471754 countsByYear W20234717542022 @default.
- W2023471754 countsByYear W20234717542023 @default.
- W2023471754 crossrefType "journal-article" @default.
- W2023471754 hasAuthorship W2023471754A5000234813 @default.
- W2023471754 hasAuthorship W2023471754A5014820840 @default.
- W2023471754 hasAuthorship W2023471754A5020023657 @default.
- W2023471754 hasAuthorship W2023471754A5023577779 @default.
- W2023471754 hasAuthorship W2023471754A5024375351 @default.
- W2023471754 hasAuthorship W2023471754A5036909379 @default.
- W2023471754 hasAuthorship W2023471754A5038978687 @default.
- W2023471754 hasAuthorship W2023471754A5040740846 @default.
- W2023471754 hasAuthorship W2023471754A5044314042 @default.
- W2023471754 hasAuthorship W2023471754A5044963611 @default.
- W2023471754 hasAuthorship W2023471754A5047244385 @default.