Peroxisomal Bifunctional Enzyme

A monomeric protein found in liver peroxisomes that contains two enzymatically active domains; an enoyl-CoA hydratase/3,2-trans-enoyl-CoA isomerase domain, and an (S)-3-hydroxyacyl-CoA dehydrogenase domain. The enzyme is stereospecific with regards to how cis and trans double bonds are metabolized. It is complemented by PEROXISOMAL MULTIFUNCTIONAL PROTEIN-2, which has the opposite stereospecificity.

Also Known As:

2-Enoyl-CoA Hydratase, 3-Hydroxyacyl-CoA Dehydrogenase-delta 3, delta 2-Enoyl-CoA Isomerase; Enoyl-CoA Hydratase-3-Hydroxyacyl-CoA Dehydrogenase; L-Bifunctional Enzyme; L-Peroxisomal Bifunctional Enzyme; Peroxisomal Hydratase-Dehydrogenase-Epimerase; Peroxisomal Multifunctional beta-Oxidation Protein; Peroxisomal Trifunctional Enzyme; Bifunctional Enzyme, L-Peroxisomal; Bifunctional Enzyme, Peroxisomal; Dehydrogenase, Enoyl-CoA Hydratase-3-Hydroxyacyl-CoA; Enoyl CoA Hydratase 3 Hydroxyacyl CoA Dehydrogenase; Hydratase-3-Hydroxyacyl-CoA Dehydrogenase, Enoyl-CoA; Hydratase-Dehydrogenase-Epimerase, Peroxisomal; L Bifunctional Enzyme; L Peroxisomal Bifunctional Enzyme; Peroxisomal Bifunctional Enzymes; Peroxisomal Hydratase Dehydrogenase Epimerase; Peroxisomal Multifunctional beta Oxidation Protein; Peroxisomal-Bifunctional Enzymes

Networked: 18 relevant articles (0 outcomes, 1 trials/studies)

Bio-Agent Context: Research Results

Experts

1.	Bjork, James A: 1 article (12/2021)
2.	Dawson, Douglas A: 1 article (12/2021)
3.	Krogstad, Jacob O: 1 article (12/2021)
4.	Wallace, Kendall B: 1 article (12/2021)
5.	Chen, Xiaoguang: 1 article (01/2021)
6.	Dobrowolski, Steven F: 1 article (01/2021)
7.	Goetzman, Eric S: 1 article (01/2021)
8.	Gorka, Joanna E: 1 article (01/2021)
9.	Lu, Jie: 1 article (01/2021)
10.	Mandel, Jordan A: 1 article (01/2021)

Related Diseases

1.	Hepatomegaly 11/01/1994 - "DHEA was fed in diet at a concentration of 0.45% for 2 weeks and livers were analyzed for hepatomegaly, peroxisome volume density, peroxisome proliferator associated Mr 80,000 polypeptide (PPA-80), and enoyl-CoA hydratase/3-hydroxyacyl-CoA dehydrogenase (PBE) mRNA. " 02/01/1990 - "In studies designed to define some of the basic mechanisms that underline the beneficial chemopreventive effects exerted by the action of this steroid, we found that the liver undergoes profound changes that result in: (i) hepatomegaly; (ii) color change from pink to mahogany; (iii) proliferation of peroxisomes; (iv) increased cross-sectional area and volume density of peroxisomes; (v) increased or decreased number of mitochondria per cell; (vi) decreased mitochondrial cross-sectional area; (vii) marked induction of the peroxisomal bifunctional protein enoyl-CoA hydratase/3-hydroxyacyl-CoA dehydrogenase; (viii) increased activities of enoyl-CoA hydratase and other peroxisomal enzymes assayed in this study, viz. " 02/01/1998 - "Deficiency of enoyl-CoA hydratase: 3-hydroxyacyl-CoA dehydrogenase (peroxisomal bifunctional enzyme), one of the enzymes of the peroxisomal beta-oxidation system, leads to clinical manifestations resembling Zellweger syndrome with hypotonia, psychomotor delay, hepatomegaly, typical facial appearance and accumulation of very long-chain fatty acids. "
2.	Hepatocellular Carcinoma (Hepatoma) 12/01/2021 - "Exposure of FaO rat hepatoma cells for 24 h to 25 μM-200 μM of the 4- and 8-carbon perfluorocarboxylic acids (PFBA and PFOA) or the 4, 6, and 8-carbon perfluorosulfonic acids (PFBS, PFHxS, and PFOS, respectively) individually caused a dose-dependent increase in PPARα-regulated expression of peroxisomal bifunctional enzyme (Ehhadh). " 07/01/2004 - "These findings were verified in an FAO rat hepatoma cell line that stably expresses PPARalpha, where the endogenous PPARalpha target genes peroxisomal bifunctional enzyme and peroxisomal 3-ketoacyl-CoA thiolase were activated up to approximately 10-20-fold by PFOS and FOSA. " 01/01/1999 - "Decreased expression of the peroxisomal bifunctional enzyme and carbonyl reductase in human hepatocellular carcinomas." 09/15/1992 - "Parallel mRNA and immunoblot analyses of the subsequent enzymes in the peroxisomal beta-oxidation pathway, enoyl-CoA hydratase/3-hydroxyacyl-CoA dehydrogenase/delta 3,delta 2-enoyl-CoA isomerase and 3-oxoacyl-CoA thiolase, showed an even stronger induction by tetradecylthioacetic acid and dexamethasone, while the counteraction by insulin was maintained in both 7800C1 hepatoma cells and hepatocytes. "
3.	Zellweger Syndrome (Zellweger's Syndrome) 02/01/1996 - "Patterns of brain dysgenesis that resemble those in the Zellweger syndrome were demonstrated in a boy with an isolated defect of the peroxisomal bifunctional enzyme. " 03/01/1987 - "Very long chain fatty acids, which accumulate in plasma and tissues in X-linked adrenoleukodystrophy (ALD), neonatal ALD, and the Zellweger cerebrohepatorenal syndrome, are degraded by the peroxisomal beta-oxidation pathway, consisting of acyl-CoA oxidase, the bifunctional enoyl-CoA hydratase/3-hydroxyacyl-CoA dehydrogenase, and beta-ketothiolase. " 02/01/1998 - "Deficiency of enoyl-CoA hydratase: 3-hydroxyacyl-CoA dehydrogenase (peroxisomal bifunctional enzyme), one of the enzymes of the peroxisomal beta-oxidation system, leads to clinical manifestations resembling Zellweger syndrome with hypotonia, psychomotor delay, hepatomegaly, typical facial appearance and accumulation of very long-chain fatty acids. "
4.	Body Weight (Weight, Body) 10/01/1994 - "Peroxisome proliferation was assessed by measuring fatty acyl-CoA oxidase (FAO) activity, peroxisomal bifunctional enzyme (PBE) quantity, and changes in liver-to-body weight ratios (LWR). " 07/01/1993 - "Induction of peroxisomal beta-oxidation was measured by changes in liver-to-body weight ratio, fatty acyl-CoA oxidase (FAO) activity, and peroxisomal bifunctional enzyme (PBE) quantity. "
5.	Liver Failure 01/01/2021 - "Mice lacking the peroxisomal bifunctional enzyme enoyl-CoA hydratase/3-hydroxyacyl CoA dehydrogenase (Ehhadh) and supplemented with the 12-carbon fatty acid lauric acid (C12) accumulate the toxic metabolite dodecanedioic acid (DDDA), which causes acute hepatocyte necrosis and liver failure. "

Related Drugs and Biologics

1.	Enzymes
2.	Enoyl-CoA Hydratase
3.	Proteins (Proteins, Gene)
4.	Steroids
5.	Acyl-CoA Oxidase
6.	Peroxisomal Bifunctional Enzyme
7.	Messenger RNA (mRNA)
8.	Fatty Acids (Saturated Fatty Acids)
9.	Isomerases
10.	Acids