Literature DB >> 894105

Hepatic lesions and hemolysis following administration of 3alpha, 7alpha, 12alpha-trihydroxy-5beta-cholestan-26-oyl taurine to rats.

R F Hanson, G C Williams, D Hachey, H L Sharp.   

Abstract

Patients with a metabolic block in the conversion of THCA into cholic acid develop cirrhosis and hemolysis, and die of hepatic failure. In these patients, THCA is largely conjugated to taurine (tauro-THCA) and excreted instead of being converted into cholic acid. In the present study, the effects of tauro-THCA on hemolysis, bile flow, and hepatic morphology were evaluated in bile fistula rats. All rats infused with tauro-THCA at rates of 0.25, 0.50 or 0.75 micronmol/min developed hemolysis with hemoglobinuria. A direct toxic effect of tauro-THCA on washed human red blood cell membranes was demonstrated at a concentration of 8 X 10(-4) M. Liver biopsy sections from rats infused for a 2 hr period with tauro-THCA were examined by electron microscopy and showed dilation of the rough endoplasmic reticulum and distortion of mitochondrial membranes. Cholestasis was not induced, since tauro-THCA actually caused a greater choleretic response for a given rate of bile salt excretion than did taurocholate. This study raises the possibility that the clinical liver disease seen in patients with a metabolic block in the conversion of THCA into cholic acid may be caused by tauro-THCA.

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Year:  1977        PMID: 894105

Source DB:  PubMed          Journal:  J Lab Clin Med        ISSN: 0022-2143


  3 in total

1.  Disturbances in bile acid metabolism of infants with the Zellweger (cerebro-hepato-renal) syndrome.

Authors:  L Monnens; J Bakkeren; G Parmentier; G Janssen; U van Haelst; F Trijbels; H Eyssen
Journal:  Eur J Pediatr       Date:  1980       Impact factor: 3.183

2.  A PEX10 defect in a patient with no detectable defect in peroxisome assembly or metabolism in cultured fibroblasts.

Authors:  S J Steinberg; A Snowden; N E Braverman; L Chen; P A Watkins; P T Clayton; K D R Setchell; J E Heubi; G V Raymond; A B Moser; H W Moser
Journal:  J Inherit Metab Dis       Date:  2008-12-25       Impact factor: 4.982

3.  Oral Cholic Acid Is Efficacious and Well Tolerated in Patients With Bile Acid Synthesis and Zellweger Spectrum Disorders.

Authors:  James E Heubi; Kevin E Bove; Kenneth D R Setchell
Journal:  J Pediatr Gastroenterol Nutr       Date:  2017-09       Impact factor: 2.839

  3 in total

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