Targeted pharmacotherapy in progressive familial intrahepatic cholestasis type 2: Evidence for improvement of cholestasis with 4-phenylbutyrate

Hepatology

Volumn 62, Issue 2, 2015, Pages 558-566

  Gonzales, Emmanuel ^a   Grosse, Brigitte ^a   Schuller, Brice ^a   Davit Spraul, Anne ^a   Conti, Filomena ^b   Guettier, Catherine ^c   Cassio, Doris ^a   Jacquemin, Emmanuel ^a  

^a UNIVERSITÉ PARIS SACLAY   (France)

^b INSERM   (France)

^c HÔPITAL SAINT ANTOINE   (France)

Author keywords

[No Author keywords available]

Indexed keywords

4 PHENYLBUTYRIC ACID; BILE ACID; BILE SALT EXPORT PUMP; GREEN FLUORESCENT PROTEIN; RIFAMPICIN; URSODEOXYCHOLIC ACID; 4-PHENYLBUTYRIC ACID; ABC TRANSPORTER; ABCB11 PROTEIN, HUMAN; ARYLBUTYRIC ACID DERIVATIVE;

ADOLESCENT; ADULT; ARTICLE; BILE ACID BLOOD LEVEL; CASE REPORT; CELLULAR DISTRIBUTION; CHILD; DRUG DOSE INCREASE; DRUG TOLERANCE; ENDOPLASMIC RETICULUM; FOLLOW UP; HUMAN; HUMAN TISSUE; INTRAHEPATIC CHOLESTASIS; LIVER INJURY; MISSENSE MUTATION; MUTANT; PRIORITY JOURNAL; PROGRESSIVE FAMILIAL INTRAHEPATIC CHOLESTASIS TYPE 2; PROSPECTIVE STUDY; PROTEIN EXPRESSION; PRURITUS; UNSPECIFIED SIDE EFFECT; ANIMAL; CELL CULTURE; CHOLESTASIS, INTRAHEPATIC; DOSE RESPONSE; DRUG ADMINISTRATION; DRUG EFFECTS; EPIDEMIOLOGY; FEMALE; GENETIC TRANSFECTION; GENETICS; MALE; MOLECULARLY TARGETED THERAPY; PATHOLOGY; PROCEDURES; RAT; TIME; TREATMENT OUTCOME;

ANIMALS; ATP-BINDING CASSETTE TRANSPORTERS; CELLS, CULTURED; CHOLESTASIS, INTRAHEPATIC; DOSE-RESPONSE RELATIONSHIP, DRUG; DRUG ADMINISTRATION SCHEDULE; FEMALE; FOLLOW-UP STUDIES; HUMANS; MALE; MOLECULAR TARGETED THERAPY; MUTATION, MISSENSE; PHENYLBUTYRATES; RATS; SAMPLING STUDIES; TIME FACTORS; TRANSFECTION; TREATMENT OUTCOME;

EID: 84938196282     PISSN: 02709139     EISSN: 15273350     Source Type: Journal    
DOI: 10.1002/hep.27767     Document Type: Article

References (28)

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