Discover Community Create You

Loading publication...

Noodle always counts identifier-free feature events for service operation. With your permission, Google Analytics can also estimate audience and engagement. Search text, publication identifiers, cookies, IP addresses and browser fingerprints are never included in Noodle's first-party event counters.

Privacy details

Article

Cholic acid therapy in Zellweger spectrum disorders

28 Jul 2016

Abstract excerpt

INTRODUCTION: Zellweger spectrum disorders (ZSDs) are characterized by a failure in peroxisome formation, caused by autosomal recessive mutations in different PEX genes. At least some of the progressive and irreversible clinical abnormalities in patients with a ZSD, particularly liver dysfunction, are likely caused by the accumulation of toxic bile acid intermediates. We investigated whether cholic acid...

Read the complete abstract on PubMed

Topics

Share this publication in a Topic to start or enrich a Post.

Share to Drug Transport and Resistance Mechanisms
Share to Liver Disease Diagnosis and Treatment
Share to Peroxisome Proliferator-Activated Receptors

Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

Source

Identifiers

PMID: 27469511
DOI: 10.1007/s10545-016-9962-9

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 8 Sept 2026.

Related research

Semantic proximity does not establish scientific evidence.

Research trail→

Click a neighbor to travelStep 1 · 12 closest

Cholic acid therapy in Zellweger spectrum disordersPMID 27469511Read ↗

Cholbam® and Zellweger spectrum disorders: treatment implementation and managementSemantic proximity Read ↗

Diagnostic challenges and disease management in patients with a mild Zellweger spectrum disorder phenotype.Semantic proximity Read ↗

IMPAIRMENT OF PEROXISOME BIOGENESIS IN THE SPECTRUM OF ZELLWEGER SYNDROME (CLINICAL CASE).Semantic proximity Read ↗

Longitudinal study of liver disease progression in the PEX1-Gly844Asp mouse model of mild Zellweger Spectrum DisorderSemantic proximity Read ↗

Cholestasis and cholestatic syndromes.Semantic proximity Read ↗

Cholestyramine Administration Induces Gut Dysbiosis and Severe Liver Injury in ApoE-/- MiceSemantic proximity Read ↗

Unbalanced lipolysis results in lipotoxicity and mitochondrial damage in peroxisome-deficient Pex19 mutants.Semantic proximity Read ↗

Novel bile salt analogs reduce lipid accumulation in liver cells with potential to treat both metabolic dysfunction-associated steatotic liver disease and Clostridioides difficile infectionSemantic proximity Read ↗

Lessons from the toxic bile concept for the pathogenesis and treatment of cholestatic liver diseases.Semantic proximity Read ↗

Hepatic dysfunction in peroxisomal disorders.Semantic proximity Read ↗

Liver disease caused by failure to racemize trihydroxycholestanoic acid: gene mutation and effect of bile acid therapy.Semantic proximity Read ↗

Genetic defects in bile acid conjugation cause fat-soluble vitamin deficiency.Semantic proximity Read ↗

⌁Select a neighboring publication to make it the new centre.