Favorable effect of 4-phenylacetate on liver functions attributable to enhanced bile salt export pump expression in ornithine transcarbamylase-deficient children

Molecular Genetics and Metabolism

Volumn 100, Issue 2, 2010, Pages 123-128

  Nagasaka, Hironori ^a   Yorifuji, Tohru ^b   Kobayashi, Kunihiko ^c   Takikawa, Hajime ^d   Komatsu, Haruki ^e   Inui, Ayano ^e   Fujisawa, Tomoo ^e   Miida, Takashi ^f   Tsukahara, Hirokazu ^g   Takatani, Tomozumi ^h   Hayashi, Hisamitsu ⁱ  

^a CHIBA CHILDREN'S HOSPITAL   (Japan)

^b KYOTO UNIVERSITY GRADUATE SCHOOL OF MEDICINE   (Japan)

^c SAPPORO HOKUYU HOSPITAL   (Japan)

^d TEIKYO UNIVERSITY SCHOOL OF MEDICINE   (Japan)

^e Eastern Yokohama Hospital   (Japan)

^f JUNTENDO UNIVERSITY   (Japan)

^g UNIVERSITY OF FUKUI HOSPITAL   (Japan)

^h CHIBA UNIVERSITY   (Japan)

ⁱ UNIVERSITY OF TOKYO   (Japan)

Author keywords

4 Phenylacetate; 4 Phenylbutyrate; Bile salt export pump; Cholestasis; Hyperammonemia; Ornithine transcarbamylase deficiency; Progressive intrahepatic cholestasis; Urea cycle defects

Indexed keywords

4 PHENYLACETIC ACID; ABC TRANSPORTER; ABC TRANSPORTER B11; ALANINE AMINOTRANSFERASE; ARGININE; ASPARTATE AMINOTRANSFERASE; BENZOIC ACID; BILE ACID; BILE SALT; BILE SALT EXPORT PUMP; GAMMA GLUTAMYLTRANSFERASE; MEMBRANE PROTEIN; MESSENGER RNA; PHENYLACETIC ACID; UNCLASSIFIED DRUG;

ADOLESCENT; ADULT; ARTICLE; BILE ACID BLOOD LEVEL; BILE SECRETION; CASE REPORT; CHILD; DIET THERAPY; FEMALE; HUMAN; HYPERAMMONEMIA; INTRAHEPATIC BILE DUCT; LIVER CELL; LIVER FUNCTION; ORNITHINE TRANSCARBAMYLASE DEFICIENCY; PRIORITY JOURNAL; PROTEIN EXPRESSION; RETROSPECTIVE STUDY; WESTERN BLOTTING;

ADENOSINE TRIPHOSPHATASES; ADOLESCENT; ARGININE; ATP-BINDING CASSETTE TRANSPORTERS; BILE ACIDS AND SALTS; CHILD, PRESCHOOL; DIET, PROTEIN-RESTRICTED; FEMALE; HUMANS; HYPERAMMONEMIA; LIVER; ORNITHINE CARBAMOYLTRANSFERASE DEFICIENCY DISEASE; PHENYLACETATES; RECEPTORS, CYTOPLASMIC AND NUCLEAR; RETROSPECTIVE STUDIES; RNA, MESSENGER; SODIUM BENZOATE;

ANIMALIA;

EID: 77951975012     PISSN: 10967192     EISSN: 10967206     Source Type: Journal    
DOI: 10.1016/j.ymgme.2010.02.008     Document Type: Article

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