Biosynthesis of very long chain fatty acids by the sciatic nerve of the rabbit

Cassagne, Claude; Darriet, D.; Bourre, Jean‐Marie

doi:10.1016/0014-5793(78)80399-8

Cited by 28 publications

(12 citation statements)

References 28 publications

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“…As well as this, a small proportion (0.5 to 3% in wild-type yeast) of VLCFA with 20 or more carbon atoms are characteristic components of all eucaryotic membranes (21,30,92,176). Yeast VLCFAs have chain lengths of 24 to 26 carbon atoms and are usually attached by amide likages to the sphingosine backbone of sphingolipids (for a review, see reference 27).…”

Section: Fatty Acid Elongasesmentioning

confidence: 79%

“…In African trypanosomes, massive amounts of 14:0 are synthesized by a type II system and used for remodeling the glycosylphosphatidylinositol anchors of their surface glycoproteins (96). The vast majority of FAS products being incorporated into the phospholipids of biological membranes comprise 16-to 18-carbon fatty acids together with a small proportion (Ͻ5%) of 26-to 30-carbon VLCFAs in eucaryotes (21,92,176). Mycobacterial meromycolic acid, on the other hand, extend to lengths of 50 or more carbon atoms.…”

Section: Chain Length Determinationmentioning

confidence: 99%

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Microbial Type I Fatty Acid Synthases (FAS): Major Players in a Network of Cellular FAS Systems

Schweizer

Hofmann

2004

Microbiol Mol Biol Rev

325

286

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The present review focuses on microbial type I fatty acid synthases (FASs), demonstrating their structural and functional diversity. Depending on their origin and biochemical function, multifunctional type I FAS proteins form dimers or hexamers with characteristic organization of their catalytic domains. A single polypeptide may contain one or more sets of the eight FAS component functions. Alternatively, these functions may split up into two different and mutually complementing subunits. Targeted inactivation of the individual yeast FAS acylation sites allowed us to define their roles during the overall catalytic process. In particular, their pronounced negative cooperativity is presumed to coordinate the FAS initiation and chain elongation reactions. Expression of the unlinked genes, FAS1 and FAS2, is in part constitutive and in part subject to repression by the phospholipid precursors inositol and choline. The interplay of the involved regulatory proteins, Rap1, Reb1, Abf1, Ino2/Ino4, Opi1, Sin3 and TFIIB, has been elucidated in considerable detail. Balanced levels of subunits α and β are ensured by an autoregulatory effect of FAS1 on FAS2 expression and by posttranslational degradation of excess FAS subunits. The functional specificity of type I FAS multienzymes usually requires the presence of multiple FAS systems within the same cell. De novo synthesis of long-chain fatty acids, mitochondrial fatty acid synthesis, acylation of certain secondary metabolites and coenzymes, fatty acid elongation, and the vast diversity of mycobacterial lipids each result from specific FAS activities. The microcompartmentalization of FAS activities in type I multienzymes may thus allow for both the controlled and concerted action of multiple FAS systems within the same cell

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Section: Fatty Acid Elongasesmentioning

confidence: 79%

Section: Chain Length Determinationmentioning

confidence: 99%

Microbial Type I Fatty Acid Synthases (FAS): Major Players in a Network of Cellular FAS Systems

Schweizer

Hofmann

2004

Microbiol Mol Biol Rev

325

286

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“…Forma tion of saturated VLCFAs. including C26:0. from [1_1 4 C] C18:0 was first demonstrated in rat sciatic nerve (11) and subsequently in normal and ALD cultured human skin fibroblasts (68). Bourre et al concluded that a single enzyme is responsible for the elongation of behenic acid (C22:0) and its monounsaturated counterpart.…”

Section: Synthesis Of Vlcfasmentioning

confidence: 99%

Dietary Management of X-Linked Adrenoleukodystrophy

Moser

Borel²

1995

Annu. Rev. Nutr.

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Adrenoleukodystrophy (ALD) is an X-linked disorder that involves mainly the nervous system white matter and adrenal cortex. It is associated with the accumulation of saturated very-long-chain fatty acids (VLCFAs), such as hexacosanoic acid (C26:0), that occurs as a result of the impaired capacity to degrade these substances, a reaction that normally takes place in the peroxisome. The VLCFAs originate from the diet and are also synthesized endogenously. Interest in dietary therapy arose from the observation that the administration of oils containing erucic and oleic acid (Lorenzo's oil), when combined with restriction of dietary intake of VLCFAs, can normalize plasma VLCFA levels in ALD patients. Clinical results in patients who are already symptomatic have been disappointing. However, preliminary data, still in need of confirmation, suggest that dietary therapy begun in asymptomatic patients can reduce the frequency and severity of later neurological disability.

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“…Besides these long-chain fatty acids, a small proportion (1-5%) of very-long-chain fatty acids (VLCFAs) 1 with lengths of 20-30 carbon atoms is usually found in eukaryotic membrane lipids (Welch and Burlingame 1973;Cassagne et al 1978;Millar and Kunst 1997;Dittrich et al 1998). In exceptional cases, VLCFAs may represent up to 30% of the total fatty acids, as in the seed oils and epicuticular waxes of certain plants (Millar and Kunst 1997).…”

Section: Introductionmentioning

confidence: 99%