Long-chain 3-hydroxy acyl-CoA dehydrogenase

Trifunctional Protein and Long-Chain 3-Hydroxy Acyl-CoA Dehydrogenase Deficiencies... 

Fig. 23.1 Body composition and lipid deposition. Data are presented as means SD. (a) There is a trend for long-chain 3-hydroxy acyl-CoA dehydrogenase (LCHAD)-deficient patients (n = 9 closed bars) to have less fat-free mass and more fat mass compared with control subjects (n = 9 open bars) when expressed as %body mass, (b) There was no difference in fat-free or fat mass expressed as mass/surface area between groups, (c) There was a trend for LCHAD-deficient patients (n = 9 closed bars) to have more extramyocellular Upid EMCL) but no difference in intramyocellular lipid IMCL) compared with...

Gillingham MB, et al. Metabolic control during exercise with and without medium-chain triglycerides (MCT) in children with long-chain 3-hydroxy acyl-CoA dehydrogenase (LCHAD) or trifunctional protein (TFP) deficiency. Mol Genet Metab. 2006 89(l-2) 58-63. 

Long-chain 3-hydroxy-acyl-CoA dehydrogenase-a/ deficiency 14.8... 

Figure 55-14 Plasma profiles of plasma acylcarnitine butyl-ester derivatives. A, Normal control. B, Propionic acidemia. C, Short-chain acyl-CoA dehydrogenase deficiency. D, Isovaleric acidemia. E, Medium-chain acyl-CoA dehydrogenase deficiency. F, Very long-chain acyl-CoA dehydrogenase deficiency. G, Long-chain L-3-hydroxy acyl-CoA dehydrogenase deficiency.The symbol marks internal standards [ Hjj-acetylcarnitine (m/z 263) [ HaJ-propionylcarnitine (m/z 277) fH ]-butyrylcarnitlne (m/z 295) pHal-octanoylcarnitine (m/z 347) [ Haj-dodecanoylcarnltine (m/z 403) [ Haj-palmitoy I carnitine (m/z 459).

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.

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