Long chain 3-hydroxyacyl-coenzyme

Treem WR et al Acute fatty liver of pregnancy and long-chain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency. Hepatology 1994 19 339. 

LCHAD long-chain 3-hydroxyacyl-coenzyme A dehydrogenase... 

Treem, W.R., Rinaldo, P., Hale, D.E., Stanley, C.A., Millington, D.S., Hyams, J.S., Jackson, S., Turnbull, D.M. Acute fatty liver of pregnancy and long-chain 3-hydroxyacyl coenzyme A dehydrogenase deficiency Hepatology 1994 19 339-345... 

Carpenter K, PoUitt RJ, Middleton B. Human liver long-chain 3-hydroxyacyl-coenzyme A dehydrogenase is a multifunctional membrane-bound beta-oxidation enzyme of mitochondria. Biochem Biophys Res Commun 1992 183 443-8. 

As noted above, there have been reports that link some cases of APLP with a defect in fatty acid metabolism in the fetus. These include fetal deficiencies of long chain 3-hydroxyacyl-coenzyme A dehydrogenase (LCHAD), carnitine-palmitoyl transferase 1 (CPT 1), and medium chain acyl-coenzyme A dehydrogenase (MCAD). The mechanism by which defective fetal fatty acid oxidation causes maternal illness is not known. However, since the fetus uses primarily glucose metabolism for its energy needs, it is likely that toxic products from the placenta, which does use fatty acid oxidation, cause the maternal liver failure. 

Long-Chain 3-Hydroxyacyl-Coenzyme A Dehydrogenase (LCHAD) Deficiency... 

Duran M, Wanders RJ, de Jager JP, Dorland L, Bruinvis L, Ketting D, et al. 3-hydroxydicarboxylic aciduria due to long-chain 3-hydroxyacyl-coenzyme a dehydrogenase deficiency associated with sudden neonatal death protective effect of medium-chain triglyceride treatment. Eur J Pediatr. 1991 150(3) 190-5. PubMed PMID 2044590, Epub 1991/01/01. eng. 

This enzyme [EC 1.1.1.35] catalyzes the reaction of an (5)-3-hydroxyacyl-CoA with NAD+ to produce a 3-oxo-acyl-CoA and NADH. The enzyme will also utilize 5-3-hydroxyacyl-A-acylthioethanolamine and 5-3-hydroxya-cylhydrolipoate as substrates. The enzyme isolated from some sources can also utilize NADP+ as the coenzyme, albeit as a weaker substrate. In addition, there is a broad specificity with respect to the acyl chain length (note that there is a long-chain-length 3-hydroxyacyl-CoA de-hydogenase [EC 1.1.1.211]). 

Rakheja D, Bennett MI, Rogers BB. Long-chain L-3-hydroxyacyl-coenzyme A dehydrogenase deficiency A molecular and biochemical review. Lab Invest 2002 82 815-24. 

He, X.-Y., Yang, S.-Y. Schulz, H. ( 992)Arch. Biochem. Biophys. 298, 527-531. Inhibition ofenoyl-CoA hydratase by long-chain L-3-hydroxyacyl-CoA and its possible effect on fatty acid oxidation. Powell, P.J., Lau, S.M., Killian, D. Thorpe, C. (1987) Biochem. 26, 3704-3710. Interaction of acyl coenzyme A substrates and analogues with pig kidney medium-chain acyl-CoA dehydrogenase. 

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