L-3-Hydroxyacyl-S-CoA

L-3-Hydroxyacyl-S-CoA + NAD+ <=> 3-Ketoacyl-S-CoA + NADH + H+ (catalyzed by 3-Hydroxyacyl-CoA Dehydrogenase)... 

L-3-Hydroxyacyl-S-CoA is an intermediate in fatty acid oxidation (Figure 18.16) that participates in the reactions below ... 

Trans- A2-Enoyl-S-CoA + H20 <=> L-3-Hydroxyacyl-S-CoA (catalyzed by Enoyl-CoA Hydratase)... 

Yan SD, Przedborski S. L-3-hydroxyacyl-CoA dehydrogenase 11 protects in a model of Parkinsoris disease. Ann Neurol 2004, 56, 51-60. 

Qi, C., Zhu, Y., Pan, J., Usuda, N., Maeda, N., Yeldandi, A. V., Rao, M. S., Hashimoto, T., and Reddy, J. K. 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 274 (1999) 15775-15780. 

He, X.-Y., Yang, S.-Y. Schulz, H. (1992) Arch. Biochem. Biophys. 298, 527-531. Inhibition of enoyl-CoA hydratase by long-chain L-3-hydroxyacyl-CoA and its possible effect on fatty acid oxidation. 

Harris, D. Das, A. (1991) Biochem. J. 280, 561-573. Control of mitochondrial ATP synthesis in heart. He, X., Yang, S. Schulz, H. (19 9) Anal. Biochem. 180, 105-109. Assay of L-3-hydroxyacyl-CoA dehydrogenase with substrates of different chain lengths. 

He, X.-Y. Yang, S.-Y. (1996) Biochemistry 35, 9625-9630. Histidine-450 is the catalytic residue of L 3-hydroxyacyl-CoA dehydrogenase associated with the large a-subunit of the multienzyme complex of fatty add oxidation from Escherichia coli. 

In the second step of the /3-oxidation cycle (Fig. 17-8a), water is added to the double bond of the tran.s-A2-enoyl-CoA to form the l stereoisomer of /3-hydroxyacyl-CoA (3-hydroxyacyl-CoA). This reaction, catalyzed by enoyl-CoA hydratase, is formally analogous to the fumarase reaction in the citric acid cycle, in which H20 adds across an a-/3 double bond (p. XXX). 

Ijlst, L., Wanders, R.J.A., Ushikubo, S, Kamijo, T. Hashimoto, T. (1994) Biochim. Biophys. Acta 1215, 347-350. Molecular basis of long chain 3-hydroxyacyl-CoA dehydrogenase deficiency identification of the major disease-mutation in the a-subunit of the mitoehondrial trifunctional protein. 

Zhang, B., Marcus, S.L., Sajjadi, F.G., Alvares, K., Reddy, J.K., Subramani, S., Rachubinski, R.A. Capone, J.R (1992). Proc. Nad Acad. Sci. U.S.A. 7541-7545. Identification of a peroxisome proliferator-responsive element upstream of the gene encoding rat peroxisomal enoyl-CoA hydratase/3-hydroxyacyl-CoA dehydrogenase. 

Suzuki, Y., Jiang, L.L., Souri, M., Miyazawa, S., Fukuda, S., Zhang, Z., Une, M., Shimozawa, N., Kondo, N., Orii, T. Hashimoto, T. (1997)J. Hum. Genet. 61, 1153-1162. D-3-hydroxy yl-CoA dehydratase/D-3-hydroxyacyl-CoA dehydrogenase bifunctional protein deficiency a newly identified peroxisomal disorder. 

Fig. 8. P-Oxidation of fatty acids in E. coli. Long-chain fatty acids are transported into the cell by FadL and converted to their CoA thioesters by FadD (not shown). The acyl-CoAs are substrates for the (1) acyl-CoA dehydrogenase (YafH) to form a trans-2-enoyl-CoA. The double bond is reduced by (2) rrans-2-enoyl-hydratase (crotonase) activity of FadB. The P-hydroxyacyl-CoA is then a substrate for the NADP -dependent dehydrogenase activity of FadB (3). A thiolase, FadA (4), releases acetyl-CoA from the P-ketoacyl-CoA to form an acyl-CoA for subsequent cycles. (5) Polyunsaturated fatty acyl-CoAs are reduced by the 2,4-dienoyl-CoA reductase (FadH). (6) FadB also catalyzes the isomerization of c/s-unsaturated fatty acids to trans. (7) The epimerase activity of FadB converts O-P-hydroxy thioesters to their L-enantiomers via the /rans-2-enoyl-CoA.

We have also measured the rates of the 8-hydroxyacyl-CoA dehydrase and 2-trans-enoyl-CoA reductase reactions using the CoA derivatives of the appropriate intermediates produced during chain elongation of 5,8-18 2, 7,10-18 2 and 8,11-18 2 (Ludwig S Sprecher, unpublished results). The 8-OH-7,10-20 2 CoA, 6-OH-9,12-20 2 CoA and 3-OH-10,13-20 2 CoA were all dehydrated at about the same rate as was found for B-OH-8,11-20 2 CoA and 3-0H-18 l CoA - i.e. 

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