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Gene Review

EHHADH  -  enoyl-CoA, hydratase/3-hydroxyacyl CoA...

Homo sapiens

Synonyms: ECHD, FRTS3, L-PBE, LBFP, LBP, ...
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Disease relevance of EHHADH

  • The human PBE is similar to rat enzyme in size (Mr, approximately equal to 79,000), isoelectric point (pI, 9.8), pH optima, molecular structure as observed by rotary shadowing, and peptide pattern on NaDodSO4/PAGE after proteolytic digestion with Staphylococcus aureus V8 protease [1].
  • CONCLUSIONS: Surgical weight loss significantly improves the degree of functional disability of morbidly obese patients suffering from LBP [2].
  • To gain insight into putative ribosomal functions of LBP we performed comparative modeling of the N-terminal domain using crystal structures of S2p from Thermus thermophilus [3].
  • It has been shown previously that morbid obesity is associated with increased frequency of LBP and that surgical weight loss improves the symptomatology [2].
  • However, there are no studies to quantitatively assess the exact degree of functional disability caused by severe obesity and the degree of improvement of LBP that follows weight loss from bariatric surgery [2].

Psychiatry related information on EHHADH

  • A higher number of years of regular leisure physical activity (RLPA) decreased the risk of LBPP during pregnancy [4].

High impact information on EHHADH

  • These results suggest that the human and rat liver PBE may be different in their amino acid sequences at their antigenic sites [1].
  • This PBE is localized exclusively in the matrix of peroxisomes in liver cells by the protein A/gold immunocytochemical method [1].
  • 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 [5].
  • In the present study, rabbit lung nuclear proteins were found to bind to two genomic elements within the 5'-flanking region of the rabbit SP-A gene, termed distal binding element (DBE; -986 to -977 base pairs) and proximal binding element (PBE; -87 to -70 base pairs) [6].
  • The underlined core sequence is an E box motif; a similar sequence (CCCTCGTG) is present within the PBE [6].

Biological context of EHHADH

  • These findings suggest that the DBE and PBE act as enhancers that interact with the same or related trans-acting proteins and serve an important role in type II cell-specific, cyclic AMP-mediated regulation of SP-A gene expression [6].
  • In type II cells transfected with fusion genes containing SP-A 5'-flanking DNA linked to the human growth hormone structural gene, mutagenesis of the DBE or PBE resulted in a marked reduction of basal and cyclic AMP-stimulated fusion gene expression [6].
  • We have isolated and characterized the expression of a cDNA clone from the sea urchin Strongylocentrotus purpuratus that encodes a protein very similar to LBP/p40, originally identified as a nonintegrin, 67-kDa laminin binding protein [7].
  • Steady-state fluorescence measurements showed that the quantum yield of the pyrene probe which is stoichiometrically attached to the active site is ca. 20% lower in the aged conjugate, pyrenebutyl-O-P(O)(O-)-Cht (PBP-Cht), than in the nonaged conjugate, pyrenebutyl-O-P-(O)(OC2H5)-Cht (PBEP-Cht) [8].
  • The Food and Drug Administration (FDA) issued a second-draft guidance in August 1999 on the subject of in vivo bioequivalence, which is based on the concepts of individual and population bioequivalence (IBE and PBE, respectively) [9].

Anatomical context of EHHADH


Associations of EHHADH with chemical compounds

  • A rapid purification method was devised in which crude extract was applied directly to a PBE 94 column after buffer exchange on a PD-10 column (Sephadex G-25 M) [15].
  • The enzyme, released from human platelets by thrombin, was purified to homogeneity by ammonium sulfate precipitation, followed by chromatography on an octyl-Sepharose column and chromatofocusing on PBE 94 [16].
  • Density functional periodic boundary condition computations (PBC-PBE/6-31G*) of the structures of (5,5) and (10,10) armchair (C2F)n fluorinated SWNTs (F-SWNTs) indicate that the pyramidalization at the fluorine-binding carbon atoms are too similar to that of CH3F to enhance the hydrogen-bond acceptor properties of fluorine significantly [17].
  • The PBE/TZVP/UAHF-PCM method has been found to describe correctly the hydrolysis energies of Fe(III), deviating about 3.0 kcal mol(-1) from experimental values [18].
  • Sialoglycoproteins of different sialic acid contents have been separated from each other by chromatofocusing on the ion exchanger PBE 94 using gradients of pH 4.00 down to pH 1.00 [19].

Other interactions of EHHADH


Analytical, diagnostic and therapeutic context of EHHADH

  • Fucosyltransferase was purified from human parotid saliva by affinity chromatography on GDP-hexanolamine Sepharose, followed by chromatofocusing on PBE 94 exchanger gel [22].
  • An improved procedure for the purification of fragment D dimer derived from crosslinked plasma fibrin is described which entails chromatofocusing chromatography using PBE 94 and polybuffer 74, and gel chromatography on Sephacryl S-300 [23].
  • After overnight food and fluid restriction, nine healthy males were examined before, during, and after lower body positive pressure (LBPP) of 11 +/- 1 mmHg (mean +/- SE) for 30 min and before, during, and after graded lower body negative pressure (LBNP) of -10 +/- 1, -20 +/- 2, and -30 +/- 2 mmHg for 20 min each [24].
  • Normal-phase HPLC was more suitable for pine bark (PBE) and tannic acid extracts analysis [25].
  • Body mass index, fasting glucose, immunoreactive insulin, insulin resistance index based on a homeostasis model assessment (HOMA-R), and total and low-density lipoprotein cholesterol were significantly higher in the HBP than the LBP subgroup (whole-body insulin resistance) [26].


  1. Purification, properties, and immunocytochemical localization of human liver peroxisomal enoyl-CoA hydratase/3-hydroxyacyl-CoA dehydrogenase. Reddy, M.K., Usuda, N., Reddy, M.N., Kuczmarski, E.R., Rao, M.S., Reddy, J.K. Proc. Natl. Acad. Sci. U.S.A. (1987) [Pubmed]
  2. The effect of surgical weight reduction on functional status in morbidly obese patients with low back pain. Melissas, J., Kontakis, G., Volakakis, E., Tsepetis, T., Alegakis, A., Hadjipavlou, A. Obesity surgery : the official journal of the American Society for Bariatric Surgery and of the Obesity Surgery Society of Australia and New Zealand. (2005) [Pubmed]
  3. Comparative modeling of the N-terminal domain of the 67kDa laminin-binding protein: implications for putative ribosomal function. Kazmin, D.A., Chinenov, Y., Larson, E., Starkey, J.R. Biochem. Biophys. Res. Commun. (2003) [Pubmed]
  4. Previous physical activity decreases the risk of low back pain and pelvic pain during pregnancy. Mogren, I.M. Scandinavian journal of public health. (2005) [Pubmed]
  5. Neuronal migration abnormality in peroxisomal bifunctional enzyme defect. Kaufmann, W.E., Theda, C., Naidu, S., Watkins, P.A., Moser, A.B., Moser, H.W. Ann. Neurol. (1996) [Pubmed]
  6. Identification of enhancers in the 5'-flanking region of the rabbit surfactant protein A (SP-A) gene and characterization of their binding proteins. Gao, E., Alcorn, J.L., Mendelson, C.R. J. Biol. Chem. (1993) [Pubmed]
  7. Characterization and localized expression of the laminin binding protein/p40 (LBP/p40) gene during sea urchin development. Hung, M., rosenthal, E., Boblett, B., Benson, S. Exp. Cell Res. (1995) [Pubmed]
  8. Conformational differences between aged and nonaged pyrenebutyl-containing organophosphoryl conjugates of chymotrypsin as detected by optical spectroscopy. Steinberg, N., van der Drift, A.C., Grunwald, J., Segall, Y., Shirin, E., Haas, E., Ashani, Y., Silman, I. Biochemistry (1989) [Pubmed]
  9. PhRMA perspective on population and individual bioequivalence. Barrett, J.S., Batra, V., Chow, A., Cook, J., Gould, A.L., Heller, A.H., Lo, M.W., Patterson, S.D., Smith, B.P., Stritar, J.A., Vega, J.M., Zariffa, N. Journal of clinical pharmacology. (2000) [Pubmed]
  10. Aldose reductase from human skeletal and heart muscle. Interconvertible forms related by thiol-disulfide exchange. Vander Jagt, D.L., Robinson, B., Taylor, K.K., Hunsaker, L.A. J. Biol. Chem. (1990) [Pubmed]
  11. Comparative effects of clofibrate on peroxisomal enzymes of human (Hep EBNA2) and rat (FaO) hepatoma cell lines. Scotto, C., Keller, J.M., Schohn, H., Dauça, M. Eur. J. Cell Biol. (1995) [Pubmed]
  12. alpha-L-Fucosidase activity and isoenzyme characteristics analyzed by chromatofocusing in normal and leukemic lymphocytes. Orlacchio, A., Maffei, C., Rambotti, P., Davis, S. Anticancer Res. (1984) [Pubmed]
  13. Effect of lower-body positive pressure on postural fluid shifts in men. Hinghofer-Szalkay, H., Kravik, S.E., Greenleaf, J.E. European journal of applied physiology and occupational physiology. (1988) [Pubmed]
  14. Effect of age and physical or fence-line boar exposure on estrus and ovulation response in prepubertal gilts administered PG600. Breen, S.M., Farris, K.L., Rodriguez-Zas, S.L., Knox, R.V. J. Anim. Sci. (2005) [Pubmed]
  15. Purification and mechanism of activation of a nerve growth factor-sensitive S6 kinase from PC12 cells. Matsuda, Y., Guroff, G. J. Biol. Chem. (1987) [Pubmed]
  16. A peptidase in human platelets that deamidates tachykinins. Probable identity with the lysosomal "protective protein". Jackman, H.L., Tan, F.L., Tamei, H., Beurling-Harbury, C., Li, X.Y., Skidgel, R.A., Erdös, E.G. J. Biol. Chem. (1990) [Pubmed]
  17. How good is fluorine as a hydrogen-bond acceptor in fluorinated single-walled carbon nanotubes? Bettinger, H.F. Chemphyschem : a European journal of chemical physics and physical chemistry. (2005) [Pubmed]
  18. Density-functional theory study of Iron(III) hydrolysis in aqueous solution. De Abreu, H.A., Guimarães, L., Duarte, H.A. The journal of physical chemistry. A, Molecules, spectroscopy, kinetics, environment & general theory. (2006) [Pubmed]
  19. Chromatofocusing of sialoglycoproteins. Burness, A.T., Pardoe, I.U. J. Chromatogr. (1983) [Pubmed]
  20. Decreased expression of the peroxisomal bifunctional enzyme and carbonyl reductase in human hepatocellular carcinomas. Suto, K., Kajihara-Kano, H., Yokoyama, Y., Hayakari, M., Kimura, J., Kumano, T., Takahata, T., Kudo, H., Tsuchida, S. J. Cancer Res. Clin. Oncol. (1999) [Pubmed]
  21. Complementation analysis of patients with intact peroxisomes and impaired peroxisomal beta-oxidation. McGuinness, M.C., Moser, A.B., Poll-The, B.T., Watkins, P.A. Biochem. Med. Metab. Biol. (1993) [Pubmed]
  22. Purification and some properties of fucosyltransferase in human parotid saliva. Tamagawa, H., Inoshita, E., Takeshita, T., Takagaki, M., Shizukuishi, S., Tsunemitsu, A. J. Dent. Res. (1987) [Pubmed]
  23. A simple chromatographic procedure for the purification of the D dimer fragment from crosslinked fibrin. Masci, P.P., Whitaker, A.N., Winzor, D.J. Anal. Biochem. (1985) [Pubmed]
  24. Influence of central venous pressure change on plasma vasopressin in humans. Norsk, P., Bonde-Petersen, F., Warberg, J. J. Appl. Physiol. (1986) [Pubmed]
  25. Analysis of condensed and hydrolysable tannins from commercial plant extracts. Romani, A., Ieri, F., Turchetti, B., Mulinacci, N., Vincieri, F.F., Buzzini, P. Journal of pharmaceutical and biomedical analysis. (2006) [Pubmed]
  26. Cellular insulin resistance in Epstein-Barr virus-transformed lymphoblasts from young insulin-resistant Japanese men. Morii, T., Ohno, Y., Hirose, H., Kawabe, H., Ogata, T., Hirao, K., Eguchi, T., Maruyama, T., Kanno, Y., Hayashi, M., Saito, I., Saruta, T. Metab. Clin. Exp. (2005) [Pubmed]
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