Gene Symbol: Ehhadh
Description: enoyl-Coenzyme A, hydratase/3-hydroxyacyl Coenzyme A dehydrogenase
Alias: 1300002P22Rik, L-PBE, LBFP, LBP, MFP, MFP1, PBFE, peroxisomal bifunctional enzyme, L-bifunctional enzyme, L-specific multifunctional beta-oxdiation protein, PBE
Species: mouse
Products:     Ehhadh

Top Publications

  1. Baes M, Gressens P, Huyghe S, De N, Qi C, Jia Y, et al. The neuronal migration defect in mice with Zellweger syndrome (Pex5 knockout) is not caused by the inactivity of peroxisomal beta-oxidation. J Neuropathol Exp Neurol. 2002;61:368-74 pubmed
    ..level of the second enzyme of the pathway, namely, the hydratase-dehydrogenase multifunctional/bifunctional enzymes MFP1/L-PBE and MFP2/D-PBE...
  2. Dirkx R, Meyhi E, Asselberghs S, Reddy J, Baes M, Van Veldhoven P. Beta-oxidation in hepatocyte cultures from mice with peroxisomal gene knockouts. Biochem Biophys Res Commun. 2007;357:718-23 pubmed better define the substrate specificities of these enzymes and in particular the elusive role of peroxisomal MFP-1, hepatocyte cultures from mice with peroxisomal gene knockouts were used to assess the consequences on substrate ..
  3. Abbott B, Wood C, Watkins A, Tatum Gibbs K, Das K, Lau C. Effects of perfluorooctanoic acid (PFOA) on expression of peroxisome proliferator-activated receptors (PPAR) and nuclear receptor-regulated genes in fetal and postnatal CD-1 mouse tissues. Reprod Toxicol. 2012;33:491-505 pubmed publisher
    ..The metabolic disruption produced by PFOA may contribute to poor postnatal survival and persistent weight deficits of CD-1 mouse neonates. ..
  4. Ding J, Loizides Mangold U, Rando G, Zoete V, Michielin O, Reddy J, et al. The peroxisomal enzyme L-PBE is required to prevent the dietary toxicity of medium-chain fatty acids. Cell Rep. 2013;5:248-58 pubmed publisher activation loop normally kept under control by dicarboxylic fatty acid degradation by the peroxisomal enzyme L-PBE. Indeed, L-pbe(-/-) mice fed coconut oil overaccumulate dicarboxylic fatty acids, which activate all fatty acid ..
  5. Chen S, Lu W, Yueh M, Rettenmeier E, Liu M, Paszek M, et al. Intestinal NCoR1, a regulator of epithelial cell maturation, controls neonatal hyperbilirubinemia. Proc Natl Acad Sci U S A. 2017;114:E1432-E1440 pubmed publisher
    ..These findings provide a mechanism of NCoR1 in intestinal homeostasis during development and provide a key link to those events that control developmental repression of UGT1A1 and hyperbilirubinemia. ..
  6. Jia Y, Qi C, Zhang Z, Hashimoto T, Rao M, Huyghe S, et al. Overexpression of peroxisome proliferator-activated receptor-alpha (PPARalpha)-regulated genes in liver in the absence of peroxisome proliferation in mice deficient in both L- and D-forms of enoyl-CoA hydratase/dehydrogenase enzymes of peroxisomal be. J Biol Chem. 2003;278:47232-9 pubmed
    ..beta-oxidation at the level of the second enzyme, enoyl-CoA hydratase/L-3-hydroxyacyl-CoA dehydrogenase (L-PBE) of the inducible pathway and D-3-hydroxyacyl-CoA dehydratase/D-3-hydroxyacyl-CoA dehydrogenase (D-PBE) of the ..
  7. Autio K, Schmitz W, Nair R, Selkälä E, Sormunen R, Miinalainen I, et al. Role of AMACR (?-methylacyl-CoA racemase) and MFE-1 (peroxisomal multifunctional enzyme-1) in bile acid synthesis in mice. Biochem J. 2014;461:125-35 pubmed publisher
    ..These results indicate that Mfe-1 can contribute to the synthesis of mature bile acids in both Amacr-dependent and Amacr-independent pathways. ..
  8. Qi C, Zhu Y, Pan J, Usuda N, Maeda N, Yeldandi A, et al. Absence of spontaneous peroxisome proliferation in enoyl-CoA Hydratase/L-3-hydroxyacyl-CoA dehydrogenase-deficient mouse liver. Further support for the role of fatty acyl CoA oxidase in PPARalpha ligand metabolism. J Biol Chem. 1999;274:15775-80 pubmed
    ..In this study, the function of enoyl-CoA hydratase/L-3-hydroxyacyl-CoA dehydrogenase (L-PBE), the second enzyme of this peroxisomal beta-oxidation system, was investigated by disrupting its gene...
  9. Ferdinandusse S, Denis S, Overmars H, Van Eeckhoudt L, Van Veldhoven P, Duran M, et al. Developmental changes of bile acid composition and conjugation in L- and D-bifunctional protein single and double knockout mice. J Biol Chem. 2005;280:18658-66 pubmed
    ..An alternative pathway for bile acid biosynthesis involving the peroxisomal L-bifunctional protein (LBP) has been proposed...

More Information


  1. Huyghe S, Casteels M, Janssen A, Meulders L, Mannaerts G, Declercq P, et al. Prenatal and postnatal development of peroxisomal lipid-metabolizing pathways in the mouse. Biochem J. 2001;353:673-80 pubmed
  2. Cook W, Jain S, Jia Y, Cao W, Yeldandi A, Reddy J, et al. Peroxisome proliferator-activated receptor alpha-responsive genes induced in the newborn but not prenatal liver of peroxisomal fatty acyl-CoA oxidase null mice. Exp Cell Res. 2001;268:70-6 pubmed
  3. Houten S, Denis S, Argmann C, Jia Y, Ferdinandusse S, Reddy J, et al. Peroxisomal L-bifunctional enzyme (Ehhadh) is essential for the production of medium-chain dicarboxylic acids. J Lipid Res. 2012;53:1296-303 pubmed publisher
    L-bifunctional enzyme (Ehhadh) is part of the classical peroxisomal fatty acid ?-oxidation pathway. This pathway is highly inducible via peroxisome proliferator-activated receptor ? (PPAR?) activation...