Excretion of 3β‐hydroxy‐5‐cholenoic and 3<i>a</i>‐hydroxy‐5<i>a</i>‐cholanoic acids in urine of infants with biliary atresia

Makino, Ikuko; Sjövall, Jan; Norman, Arne; Strandvik, Birgitta

doi:10.1016/0014-5793(71)80047-9

Cited by 123 publications

(34 citation statements)

References 5 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…In the fetal and neonatal period, 3ghydroxy-5-cholenoic acid and LCA are synthesized as primary bile acids in addition to CA and CDCA (3,16). The taurine conjugated bile acids are more predominant than glycine conjugated bile acids (5,7,19), and the ratio is later reversed.…”

mentioning

confidence: 99%

Studies on the Conjugating Activity of Bile Acids in Children

Niijima

1985

Pediatr Res

View full text Add to dashboard Cite

show abstract

mentioning

confidence: 99%

Studies on the Conjugating Activity of Bile Acids in Children

Niijima

1985

Pediatr Res

View full text Add to dashboard Cite

show abstract

“…The compound 3b is thought to be a precursor for 3$-hydroxy-5-cholen-24-oic acid (lb), which was first found in the urine of a neonate with congenital biliary atresia. 17 Other compounds (3a, 3c, 3e, 3g, 3h) are selected for the biological study with rat liver. The compounds 4 and 5 are the biologically possible intermediates from the corresponding 5/i-cholestan-26-oic acids (3) in the $-oxidation step.…”

Section: Resultsmentioning

confidence: 99%

Capillary Gas Chromatographic Determination of C27-Bile Acids in Biological Samples and Its Application to the Urine of a Patient with Zellweger Syndrome

et al. 1996

View full text Add to dashboard Cite

Gas chromatographic determination of C27-bile acids was investigated for the study of bile acid biosynthesis and related congenital disorders. The intermediate C27-bile acids in the /3-oxidation step and related compounds were conveniently synthesized from the corresponding C24-bile acids as standard samples. The separation of methyl ester-trimethylsilyl ether (Me-TMS) and methyl ester-dimethylethylsilyl ether (Me-DMES) derivatives of the C27-bile acids was carried out by capillary gas chromatography.Then methylene units of the above derivatives were measured. The Me-DMES derivatives were more efficient for the separation of C27-bile acids than the Me-TMS derivatives. The calibration curves for the Me-DMES derivatives of C27-bile acids showed good linearities over the range of 5 -200 ng; the detection limits were ca. 0.5 ng (S/N=5).The relative recoveries from biological samples were 93 -105% for all C27-bile acids using 24-nor-chenodeoxycholic acid as an internal standard. An application to the diagnosis of Zellweger syndrome is also discussed.

show abstract

“…It is of interest to know whether or not unconjugated 3$-OH is present in infants with cholestatis, because unsaturated monohydroxy bile acids have been presumed to be a possible cause of idiopathic infantile obstructive cholangiopathy (Jenner and Howard 1975). A few reports have proven the presence of 3$-OH in serum or urine of cholestatic infants (Makino et al 1971; Deleze and Paumgartner 1977;Tazawa and Konno 1982), but there is no information about its conjugated forms. We detected its conjugates in the sulfated fraction in the specimens obtained from cholestatic infants but not its unconjugate, although the detection limit of 3$-OH unconjugate is higher than those of its conjugates.…”

Section: Discussionmentioning

confidence: 99%