ArticleThe Biochemical journal2002
The role of alpha-methylacyl-CoA racemase in bile acid synthesis.
Article in The Biochemical journal, 2002. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 12 papers.
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Who cites it
12 citing papers in PubMed, 38 citations in OpenAlex.
- Localization of MCT2 at peroxisomes is associated with malignant transformation in prostate cancer.Journal of cellular and molecular medicine · 2015Article
- High-throughput screen identifies novel inhibitors of cancer biomarker α-methylacyl coenzyme A racemase (AMACR/P504S).Molecular cancer therapeutics · 2011Article
- Non-synonymous variants in the AMACR gene are associated with schizophrenia.Schizophrenia research · 2010Article
- Biochemistry and genetics of inherited disorders of peroxisomal fatty acid metabolism.Journal of lipid research · 2010Review
- Major biliary bile acids of the medaka (Oryzias latipes): 25R- and 25S-epimers of 3alpha,7alpha,12alpha-trihydroxy-5beta-cholestanoic acid.Zoological science · 2010Article
- Fine-scale variation and genetic determinants of alternative splicing across individuals.PLoS genetics · 2009Article
- Bile acids: the role of peroxisomes.Journal of lipid research · 2009Review
- Cloning and characterization of alpha-methylacyl coenzyme A racemase from Gordonia polyisoprenivorans VH2.Applied and environmental microbiology · 2008Article
- Mechanisms of disease: Inborn errors of bile acid synthesis.Nature clinical practice. Gastroenterology & hepatology · 2008Review
- Alpha-methylacyl-CoA racemase (AMACR/P504S) protein expression in urothelial carcinoma of the upper urinary tract correlates with tumour progression.Virchows Archiv : an international journal of pathology · 2006Article
- Detection of alpha-methylacyl-coenzyme-A racemase transcripts in blood and urine samples of prostate cancer patients.Molecular diagnosis & therapy · 2006Article
- Elevated alpha-methylacyl-CoA racemase enzymatic activity in prostate cancer.The American journal of pathology · 2004Article
Corrections and comments
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Authors and funding
5 authors at 3 institutions in 3 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
According to current views, the second peroxisomal beta-oxidation pathway is responsible for the degradation of the side chain of bile acid intermediates. Peroxisomal multifunctional enzyme type 2 [peroxisomal multifunctional 2-enoyl-CoA hydratase/(R)-3-hydroxyacyl-CoA dehydrogenase; MFE-2] catalyses the second (hydration) and third (dehydrogenation) reactions of the pathway. Deficiency of MFE-2 leads to accumulation of very-long-chain fatty acids, 2-methyl-branched fatty acids and C(27) bile acid intermediates in plasma, but bile acid synthesis is not blocked completely. In this study we describe an alternative pathway, which allows MFE-2 deficiency to be overcome. The alternative pathway consists of alpha-methylacyl-CoA racemase and peroxisomal multifunctional enzyme type 1 [peroxisomal multifunctional 2-enoyl-CoA hydratase/(S)-3-hydroxyacyl-CoA dehydrogenase; MFE-1]. (24E)-3alpha,7alpha,12alpha-Trihydroxy-5beta-cholest-24-enoyl-CoA, the presumed physiological isomer, is hydrated by MFE-1 with the formation of (24S,25S)-3alpha,7alpha,12alpha,24-tetrahydroxy-5beta-cholestanoyl-CoA [(24S,25S)-24-OH-THCA-CoA], which after conversion by a alpha-methylacyl-CoA racemase into the (24S,25R) isomer can again be dehydrogenated by MFE-1 to 24-keto-3alpha,7alpha,12alpha-trihydroxycholestanoyl-CoA, a physiological intermediate in cholic acid synthesis. The discovery of the alternative pathway of cholesterol side-chain oxidation will improve diagnosis of peroxisomal deficiencies by identification of serum 24-OH-THCA-CoA diastereomer profiles.
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.