scholarly journals Cholic acid mediates negative feedback regulation of bile acid synthesis in mice

2002 ◽  
Vol 110 (8) ◽  
pp. 1191-1200 ◽  
Author(s):  
Jia Li-Hawkins ◽  
Mats Gåfvels ◽  
Maria Olin ◽  
Erik G. Lund ◽  
Ulla Andersson ◽  
...  
Keyword(s):  
Bile Acid ◽  
Cholic Acid ◽  
Negative Feedback ◽  
Feedback Regulation ◽  
Acid Synthesis ◽  
Bile Acid Synthesis ◽  
Negative Feedback Regulation

scholarly journals Loss of Nuclear Receptor SHP Impairs but Does Not Eliminate Negative Feedback Regulation of Bile Acid Synthesis

2002 ◽  
Vol 2 (6) ◽  
pp. 713-720 ◽  
Author(s):  
Thomas A Kerr ◽  
Shigeru Saeki ◽  
Manfred Schneider ◽  
Karen Schaefer ◽  
Sara Berdy ◽  
...  
Keyword(s):  
Bile Acid ◽  
Nuclear Receptor ◽  
Negative Feedback ◽  
Feedback Regulation ◽  
Acid Synthesis ◽  
Bile Acid Synthesis ◽  
Negative Feedback Regulation

scholarly journals Cholic acid for primary bile acid synthesis defects: a life-saving therapy allowing a favorable outcome in adulthood

2018 ◽  
Vol 13 (1) ◽  
Author(s):  
Emmanuel Gonzales ◽  
Lorenza Matarazzo ◽  
Stéphanie Franchi-Abella ◽  
Alain Dabadie ◽  
Joseph Cohen ◽  
...  
Keyword(s):  
Bile Acid ◽  
Cholic Acid ◽  
Acid Synthesis ◽  
Bile Acid Synthesis ◽  
Favorable Outcome ◽  
Life Saving ◽  
Primary Bile Acid

scholarly journals Atypical clinical presentation and successful treatment with oral cholic acid of a child with defective bile acid synthesis due to a novel mutation in the HSD3B7 gene

2017 ◽  
Vol 9 (3) ◽  
Author(s):  
Grazia Bossi ◽  
Giuseppe Giordano ◽  
Gaetana Anna Rispoli ◽  
Giuseppe Maggiore ◽  
Mauro Naturale ◽  
...  
Keyword(s):  
Bile Acid ◽  
Replacement Therapy ◽  
Cholic Acid ◽  
Novel Mutation ◽  
Failure To Thrive ◽  
Acid Synthesis ◽  
Bile Acid Synthesis ◽  
Ionization Mass ◽  
Fast Atom Bombardment Ionization ◽  
Atypical Clinical Presentation

We report definitive diagnosis and effective treatment with oral cholic acid in one Italian male child affected by 3β- hydroxy-Δ5-C27-steroid dehydrogenase (3β- HSD) deficiency. He presented with failure to thrive, hepatomegaly and multiple cystic images in kidneys; no biochemical evidence of cholestasis. Large amounts of bile acid metabolites was detected in urine by fast atom bombardment ionization mass spectrometry (FAB-MS). <em>HSDH3B7</em> gene analysis identified one mutation in intron 4, at nucleotide 432, G&gt;A substitution that has never been reported before.The replacement therapy with oral cholic acid started early after the diagnosis and is still ongoing. Three years later hepatomegaly is no longer evident, liver function is normal and the child is growing regularly. In our experience, clinical features of 3β-HSD deficiency can be very poor and even cholestasis can lack at diagnosis. Early replacement therapy with cholic acid is safe and leads to clinical and biochemical control of the disease.

scholarly journals Bile acid-induced negative feedback regulation of the human ileal bile acid transporter

Hepatology ◽  
2004 ◽  
Vol 40 (1) ◽  
pp. 149-156 ◽  
Author(s):  
Ezequiel Neimark ◽  
Frank Chen ◽  
Xiaoping Li ◽  
Benjamin L. Shneider
Keyword(s):  
Bile Acid ◽  
Negative Feedback ◽  
Feedback Regulation ◽  
Negative Feedback Regulation ◽  
Bile Acid Transporter ◽  
Acid Transporter

scholarly journals Feedback regulation of bile acid synthesis in primary human hepatocytes: Evidence that CDCA is the strongest inhibitor

Hepatology ◽  
2003 ◽  
Vol 38 (4) ◽  
pp. 930-938 ◽  
Author(s):  
Ewa Ellis ◽  
Magnus Axelson ◽  
Anna Abrahamsson ◽  
Gösta Eggertsen ◽  
Anders Thörne, ◽  
...  
Keyword(s):  
Bile Acid ◽  
Feedback Regulation ◽  
Acid Synthesis ◽  
Bile Acid Synthesis ◽  
Human Hepatocytes ◽  
Primary Human Hepatocytes

Negative feedback regulation of the ileal bile acid transport system in rodents

1993 ◽  
Vol 104 (1) ◽  
pp. 38-46 ◽  
Author(s):  
Jan Lillienau ◽  
Diane L. Crombie ◽  
Jorge Munoz ◽  
Sarah J. Longmire-cook ◽  
Lee R. Hagey ◽  
...  
Keyword(s):  
Bile Acid ◽  
Transport System ◽  
Negative Feedback ◽  
Feedback Regulation ◽  
Acid Transport ◽  
Negative Feedback Regulation ◽  
Bile Acid Transport
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