Fatty aldehyde dehydrogenase

Fatty aldehyde dehydrogenase (FALDH) and Fatty alcohohNAD oxidoreductase (FAO) Sjogren Larsson syndrome... 

FALDH Fatty aldehyde dehydrogenase and fatty alcohol NAD-oxidoreducease phosphate oxidoreductase... 

Singer, M.E. and W.R. Finnerty. 1985a. Fatty aldehyde dehydrogenases in Acineto-bacter sp. strain HOl-N role in hexadecane and hexadecanol metabolism. /. 

Of the deficits unrelated to the glycolipids which show an abundant clinical literature but less interest manifested by scientists in the field of lipidomics are ceroid lipofuscinosis (the deficiency in palmitoyl-protein thioesterase (PPT)) and the Sjogren-Larsson syndrome, a defect of ALDH3A2 gene which encodes fatty aldehyde dehydrogenase (FALDH). It is pertinent to question the reason for such differences between clinical and basic scientific interests reflected in literature citations. 

Sjogren- Larson fatty aldehyde dehydrogenase Fatty alcohol FALDH3 1 12000 north-east Sweden GC assay of plasma aUphatic alcool... 

The syndrome is caused by a particular mutation in the gene encoding fatty aldehyde dehydrogenase (gene FALDH3A2). About 1.3% of the population of northern Sweden is heterozygous for the defective gene. 

Fatty alcohohNAD oxidoreductase is a complex enzyme which consists of two separate proteins that sequentially catalyze the oxidation of fatty alcohol to fatty aldehyde and to fatty acid that is to say a fatty alcohol dehydrogenase and a fatty aldehyde dehydrogenase (FALDH) activity. Sjogren-Larsson cells were selectively deficient in the FALDH component and had normal activity of fatty alcohol dehydrogenase. Intact fibroblast oxidized octadecanol to fatty acid at <10% of the normal rate but oxidized octadecanal normally confirming that FALDH is specifically affected (Rizzo and Craft, 1991). 

Rizzo W.B., Craft D.A., Sjogren-Larsson syndrome. Deficient activity of the fatty aldehyde dehydrogenase component of fatty alcohol NAD+ oxidoreductase in cultured fibroblasts, The Journal of clinical investigation 88 (1991) 1643-1648. 

Willemsen M.A., Cruysberg J.R., Rotteveel J.J., Aandekerk A.L., Van Domburg P.H., Deutman A.F., Juvenile macular dystrophy associated with deficient activity of fatty aldehyde dehydrogenase in Sjogren-Larsson syndrome, American journal of ophthalmology 130 (2000) 782-789. 

In brief, important milestones were proofs for monooxygenase, fatty alcohol oxidase, fatty aldehyde dehydrogenase, and of glycosyltransferase, acetyl-transferase, and acetylesterase more information is given in [35]. 

Fig. 7. Branch point between fermentation and respiration. At low pyruvate flux, the low of the Pdh complex for pyruvate results in oxidative decarboxylation to form acetyl CoA and NADH. The acetyl CoA can then can go into energy generation (via respiration) or fatty acid synthesis. At high glycolytic flux, pyruvate accumulates, and the higher of Pdc favors acetaldehyde formation and ethanol production. Accumulation of acetate can interfere with mitochondrial function. Pyk Pyruvate kinase Pdh pyruvate dehydrogenase Pdc pyruvate decarboxylase Aid (Dha) aldehyde dehydrogenase Adh alcohol dehydrogenase Acs acetyl CoA synthetase. (Taken from Postma et al. [169])...

The finding of formyl-CoA, and not formate, as end-product of the a-oxidation process directly suggested that the second product might be a fatty aldehyde and not a fatty acid as assumed till then. GC-analysis of the lipophilic products formed by peroxisomes incubated with 2-hydroxy-3-metiiylhexadecanoyl-CoA showed an unknown metabolite, co-eluting with synthetic 2-methylpentadecanal. If NAD was present, the aldehyde was converted almost completely into 2-methylpentadecanoic acid, presumably by a peroxisomal aldehyde dehydrogenase. ... 

I. Long-chain fatty acid peroxidase II. Aldehyde dehydrogenase... 

The aldehyde is oxidized via the enzyme aldehyde dehydrogenase to yield fatty acid, which can be further... 

Methods w ere determined for the preparation of cell extracts and membranes. Assays w ere set up for o>-oxidation enzymes (cytochrome P-450 hydroxylase, cviochrome P-450 reductase, alcohol oxidase, aldehyde dehydrogenase) and p-oxidation enz>mes (acyl CoA oxidase, hydratase). Conditions were established for cell growth, and induction of fatty acid oxidising enzymes, under laboratory conditions using C. cloacae parent and mutant strains. 

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