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1 and severity of neuropathy and the levels of very-long-chain 1-deoxyceramides such as C24 (P < 0.05),
2 romoter between the two human genes encoding very long chain acyl-CoA dehydrogenase (VLCAD) and posts
3 ferase, very long chain acyl-CoA synthetase, very long chain acyl-CoA dehydrogenase) in livers of the
4 re we provide evidence that in this organism very long chain acyl-CoA esters are hydrolyzed by the Px
5 raphy and demonstrated a 10-fold increase in very long chain acyl-CoA specific activity (C24:0/C16:0)
6 hibited markedly decreased expression of the very long chain acyl-CoA synthase-related gene (VLACSR),
7 cell extracts suggested that FATP1 exhibits very long chain acyl-CoA synthetase (ACS) activity and t
8 Members of the fatty acid transport protein/very long chain acyl-CoA synthetase (FATP/Acsvl) family
11 , liver carnitine palmitoyl-CoA transferase, very long chain acyl-CoA synthetase, very long chain acy
12 nthetase activity (C16:0), characteristic of very long chain acyl-CoA synthetases, whereas both mutan
14 in skin homeostasis, which may be due to its very long chain acyl-coenzyme A synthetase activity.
15 t exogenous long-chain fatty acids, and have very long-chain acyl CoA synthetase activities that were
17 though many patients have been found to have very long-chain acyl-CoA dehydrogenase (VLCAD) deficienc
19 long-chain acyl-CoA dehydrogenase (LCAD) and very long-chain acyl-CoA dehydrogenase revealed that 5-t
20 unsaturated acyl-CoAs are poor substrates of very long-chain acyl-CoA dehydrogenase when compared wit
22 ls suggest that, in contrast to yeast cells, very long-chain acyl-CoA esters are transported into per
23 s been associated with decreased peroxisomal very long-chain acyl-CoA synthetase (VLCS) activity and
24 creased VLCFA beta-oxidation and peroxisomal very long-chain acyl-CoA synthetase (VLCS) activity.
26 inked adrenoleukodystrophy, are activated by very long-chain acyl-CoA synthetase (VLCS) normally foun
29 002 is a prodrug that requires activation by very long-chain acyl-CoA synthetase-1 (ACSVL1) to modula
37 trate specificity, it appears that ACAD9 and very-long-chain acyl-CoA dehydrogenase are unable to com
39 istribution and gene regulation of ACAD9 and very-long-chain acyl-CoA dehydrogenase identify the pres
40 function that is highly homologous to human very-long-chain acyl-CoA dehydrogenase was identified by
41 Gel filtration analysis indicated that, like very-long-chain acyl-CoA dehydrogenase, ACAD-9 is a dime
44 decreased ability of cells to activate (via very-long-chain acyl-coenzyme A synthetase [VLCS]) and s
49 (N-palmitoyl sphingosine) and nonsaturated, very long chain C24:1 ceramide (N-nervonoyl sphingosine)
51 hat confluence caused selective increases in very long chain ceramide C(24:1) (370 +/- 54%) and C(24:
52 bases in blood, accompanied by increases in very long chain ceramide species, and have defective lym
53 d cellular levels of specific long-chain and very long-chain ceramide species relative to vector cont
56 Stress increased the amounts of long- and very long-chain ceramides in HEK293 cells, and this was
58 terestingly, elevated levels of only certain very long-chain ceramides were evident in V5-aSMase(S508
60 ication of several endogenous long-chain and very-long-chain ceramides using two nonphysiological odd
61 s in the retina with special emphasis on the very-long-chain-containing saturated (VLC-FA) and polyun
63 d seed coats with a severalfold reduction in very long chain dicarboxylic acid and omega-hydroxy fatt
64 with FA, the rate of flip-flop of saturated very long chain FA (C20:0, C:22:0, and C24:0) was shown
65 carboxylase, inhibited only elongation into very long chain FAs (>or=20 carbons) but not synthesis o
66 ptor CD36 is required both for the uptake of very long chain FAs (VLCFAs) in cultured cells and the a
69 ed to provide insight into the regulation of very long chain fatty acid (VLCFA) biosynthesis in Brass
70 letion of ALD gene (ABCD1) and the resultant very long chain fatty acid (VLCFA) derangement has drama
73 ese studies reveal roles for Rvs161p and the very long chain fatty acid elongase, Sur4p, in the local
75 ,3-enoyl-CoA reduction reactions in long and very long chain fatty acid elongation, respectively.
76 n precursor, suggesting that it functions in very long chain fatty acid hydroxylation using an altern
77 tly linked to the (omega-1)-hydroxy group of very long chain fatty acid in bradyrhizobial lipid A.
78 Although FATP4 deficiency primarily affected very long chain fatty acid metabolism, mutant fibroblast
80 CoA synthetase that preferentially activates very long chain fatty acid substrates, such as C24:0, to
81 3-ketoacyl-CoA synthase which is involved in very long chain fatty acid synthesis in vegetative tissu
82 r C18:1, suggesting that in vivo, defects in very long chain fatty acid uptake may underlie the skin
83 oblasts, FATP4 is the major enzyme producing very long chain fatty acid-CoA for lipid metabolic pathw
87 cid elongases that catalyze the synthesis of very long chain fatty acids (C24 to C26) required for ce
88 n of VLCFA synthesizing enzymes, elongase of very long chain fatty acids (ELOVLs) (1 and 3) in both c
89 Abnormalities in the transport of saturated very long chain fatty acids (VLCFA; >C18:0) contribute t
90 mal disorder with impaired beta-oxidation of very long chain fatty acids (VLCFAs) and reduced functio
91 adrenoleukodystrophy is the accumulation of very long chain fatty acids (VLCFAs) due to impaired per
92 he bubblegum mutant shows elevated levels of very long chain fatty acids (VLCFAs), as seen in the hum
95 ngation pathway responsible for formation of very long chain fatty acids (VLCFAs, or fatty acids with
99 nvolved in the peroxisomal beta-oxidation of very long chain fatty acids and are associated with defe
100 contain unique lipids such as mycolic acids, very long chain fatty acids and multimethyl-branched fat
101 Cuticular waxes are complex mixtures of very long chain fatty acids and their derivatives that c
105 mical parameters, including accumulations of very long chain fatty acids in plasma and deficient eryt
107 miR-219 and its target ELOVL7 (elongation of very long chain fatty acids protein 7) were identified a
109 longation systems that produce the 26-carbon very long chain fatty acids that are precursors for cera
111 dehydratases are required for elongation of very long chain fatty acids, and HACD1 has a role in ear
112 golipids, substantial levels of hydroxylated very long chain fatty acids, and the full complement of
113 lar endothelial cells causes accumulation of very long chain fatty acids, but much later than the imm
116 in the expression of the gene elongation of very long chain fatty acids-like 2, an enzyme needed for
117 ulin-induced gene 2a, Insig2a, elongation of very long chain fatty acids-like 3, Elovl3 and sterol 12
118 A Met299Val variant in the elongation of very long chain fatty acids-like 4 (ELOVL4) gene was sig
131 (VLCFAs) and reduced function of peroxisomal very long chain fatty acyl-CoA synthetase (VLCS) that le
133 ption mutant (DeltafoxA) was eliminated on a very long-chain fatty acid (C(22:1)), growth was only pa
134 classes on the stem and leaf, except in the very long-chain fatty acid (VLCFA) class wherein acids l
135 In this study, we investigated the roles of very long-chain fatty acid (VLCFA) synthesis by fatty ac
137 ecrease in peroxisomal VLCS activity and the very long-chain fatty acid accumulation in the yeast FAT
138 nted the defects in fatty acid transport and very long-chain fatty acid activation associated with a
140 transport protein 4 (FATP4), which each have very long-chain fatty acid acyl-CoA synthetase (VLCFA-AC
141 phingolipid pathway, such as deletion of the very long-chain fatty acid elongase, Sur4, suppress the
144 other acyl-CoA synthetases are necessary for very long-chain fatty acid metabolism in Neuro2a cells.
146 f the ACC and elevation of the ceramides and very long-chain fatty acid syntheses with normal cell-cy
153 defect in synthesis of unsaturated long and very long-chain fatty acids (LCFAs and VLCFAs) and deple
154 Sphingolipids are synthesized de novo from very long-chain fatty acids (VLCFA) and sphingoid long-c
155 bers of this group are capable of activating very long-chain fatty acids (VLCFA), one is a bile acid-
157 re of X-ALD is increased levels of saturated very long-chain fatty acids (VLCFA; carbon chains of 22
159 (del/del) mice revealed a global decrease in very long-chain fatty acids (VLFAs) (i.e., carbon chain
160 X-ALD is characterized by an accumulation of very long-chain fatty acids and partially impaired perox
161 possibility that intermediate metabolites of very long-chain fatty acids and peroxisomal beta-oxidati
162 p and Elo3p are inviable confirming that the very long-chain fatty acids are essential for cellular f
163 tol-ceramides [IPC, MIPC, and M(IP)(2)C] and very long-chain fatty acids C(24) and C(26) declined sha
164 ssion pattern and ability of BG1 to activate very long-chain fatty acids implicates this enzyme in th
165 icantly inhibited the omega-hydroxylation of very long-chain fatty acids in cultured human keratinocy
166 rely impaired as degradation of unesterified very long-chain fatty acids in X-ALD and is abolished in
169 and Elo3p are required for synthesis of the very long-chain fatty acids, and mutants lacking both El
170 of linoleic acid, odd-chain fatty acids, and very long-chain fatty acids, was associated with lower i
172 l membrane proteins involved in transport of very long-chain fatty acids, which are a unique componen
173 NA library, we have identified elongation of very long-chain fatty acids-like 1 (ELOVL1) and fatty ac
179 long-chain fatty acids and the activation of very long-chain fatty acids; these activities intrinsic
180 ble exceptions, the fatty acid transport and very long-chain fatty acyl-CoA synthetase activities wer
181 ates, the levels of fatty acid accumulation, very long-chain fatty acyl-CoA synthetase activities, an
182 coenzyme A derivatives using long-chain and very-long chain fatty acids, bile acids and bile acid pr
183 ts, led us to discover that BacA affects the very-long-chain fatty acid (27-OHC28:0 and 29-OHC30:0) c
185 and Schefflera elegantissima) contained only very-long-chain fatty acid (VLCFA) derivatives such as a
187 chological, electrophysiological, and plasma very-long-chain fatty acid (VLCFA) measurements were use
188 se complex are required for the synthesis of very-long-chain fatty acid (VLCFA) precursors of cuticul
189 reviously undescribed desaturase activity on very-long-chain fatty acid (VLCFA) substrates and exhibi
190 slation initiation factor 2B (eIF2B) and the very-long-chain fatty acid (VLCFA) synthesis keto-reduct
192 and B. abortus are unusually modified with a very-long-chain fatty acid (VLCFA; C > or = 28) and we d
193 ectrometry (LC/MS) method for long-chain and very-long-chain fatty acid analysis and its application
194 not ZS, fibroblasts there was an increase in very-long-chain fatty acid beta-oxidation and plasmaloge
195 X-ALD fibroblasts synergistically increased very-long-chain fatty acid beta-oxidation, indicating th
197 its production of a substantial reduction of very-long-chain fatty acid levels in the brain and adren
198 drenoleukodystrophy (X-ALD) is a disorder of very-long-chain fatty acid metabolism, adrenal insuffici
201 distribution of LPS fatty acids, including a very-long-chain fatty acid thought to be unique to the a
203 he PM showed an enrichment of 2-hydroxylated very-long-chain fatty acid-containing GIPCs and polyglyc
204 ision, suggesting that enhanced synthesis of very-long-chain fatty acid/trihydroxy LCB ceramides prom
205 Individuals with ABCD1 mutations accumulate very-long-chain fatty acids (VLCFA) (carbon length >22).
207 X-ALD) is associated with elevated levels of very-long-chain fatty acids (VLCFA; C(>22:0)) that have
211 des accumulation of peroxisomal educts (like very-long-chain fatty acids [VLCFAs] or branched-chain f
212 flammatory demyelination in the brain, where very-long-chain fatty acids accumulate within phospholip
213 1/lpcat2 mutant showed increased contents of very-long-chain fatty acids and decreased PUFA in TAG an
214 lopmental and environmental requirements for very-long-chain fatty acids and flavonoids, whose synthe
215 ACC (ScACC) is crucial for the production of very-long-chain fatty acids and the maintenance of the n
216 This led to a significant increase in the very-long-chain fatty acids C24 and C26 in the cuticular
218 n GIPCs and sterols and suggested a role for very-long-chain fatty acids in the interdigitation betwe
219 mutations in ABCD1 lead to incorporation of very-long-chain fatty acids into phospholipids, we separ
220 litate the transfer of long-chain as well as very-long-chain fatty acids into the apoplast, depending
223 have shown that larval oenocytes synthesize very-long-chain fatty acids required for tracheal waterp
224 an acyl CoA synthetase activity specific for very-long-chain fatty acids suggesting this protein play
225 (CER6) is necessary for the biosynthesis of very-long-chain fatty acids with chain lengths beyond C(
227 of Elovl proteins that mediate elongation of very-long-chain fatty acids, block or dramatically slow
228 ngation of not only linear but also branched very-long-chain fatty acids, leading to production of th
229 n cause accumulation of unbranched saturated very-long-chain fatty acids, particularly in brain and a
230 pecies with C16 fatty acids rather than with very-long-chain fatty acids, which are more commonly enr
231 ndensing enzyme involved in the synthesis of very-long-chain fatty acids, which are precursors of epi
232 ng patterns of saturated and monounsaturated very-long-chain fatty acids, with the observed pattern c
233 ingle allelic mutations in the elongation of very-long-chain fatty acids-like 4 (ELOVL4), whereas rec
238 ed levels of and increased beta-oxidation of very-long-chain fatty acids; increased expression of the
239 3 (At1g19260)-encoded ceramide synthases use very-long-chain fatty acyl-CoA and trihydroxy LCB substr
240 oxidation chain shortening of long-chain and very-long-chain fatty acyl-coenzyme (CoAs), long-chain d
241 vity of class II ceramide synthases that use very-long-chain fatty acyl-coenzyme A and trihydroxy LCB
242 t jojoba FAO and FADH proteins are active on very-long-chain fatty alcohol and fatty aldehyde substra
243 gest that jojoba FAO and FADH constitute the very-long-chain fatty alcohol oxidation pathway that is
244 Upon germination, WE hydrolysis releases very-long-chain fatty alcohols, which must be oxidized t
245 ax profile (especially on leaves) toward the very-long-chain free fatty acids tetracosanoic acid (C(2
246 pressed in photoreceptor cells and generates very long chain (>/=C28) polyunsaturated fatty acids inc
247 isomal functions, including the breakdown of very long-chain (>20 carbons) fatty acids, phytanic acid
248 ancer suggest that 1-deoxysphingolipids, the very-long-chain in particular, play a role as molecular
249 istics suggest that it may interact with the very long chain intermediates in mycolic acid biosynthes
250 he other hand, was missing O-antigens of the very long chain length, indicating the Wzz2 protein is r
251 ed with other fatty acids and because of its very long chain length, the effect of dietary behenic ac
252 responsible for the expression of different very long chain lengths despite high sequence homology.
253 out for long chain palmitic acid (C16:0) and very long chain lignoceric acid (C24:0) as well as for A
254 ut for long chain (palmitic acid, C16:0) and very long chain (lignoceric acid, C24:0) fatty acids as
255 suppressor demonstrates that low amounts of very long chain lipids are sufficient for pollen hydrati
257 Mutations in Arabidopsis CER genes eliminate very long chain lipids from the cuticle surface and, in
258 e display massive increases in a rare set of very long chain LPS lipids that have been previously rep
260 and a dramatic increase in the percentage of very-long-chain monounsaturated fatty acids in the acyl-
261 whether red blood cell (RBC) long-chain and very-long-chain MUFAs are associated with risk of CAD in
263 ional enzyme that catalyzes the synthesis of very long chain multiple methyl branched fatty acids cal
267 Higher proportions of certain VLC n-3 and very long-chain n-6 PUFAs in plasma phospholipids at age
268 he effect and the mechanism of action of the very-long-chain n-3 (omega-3) polyunsaturated fatty acid
270 feasibility for CEC-ESI-MS analysis of these very long chain nonionic surfactants for the first time.
271 in place of the proximal glucosamine, and a very long chain omega-1 hydroxy fatty acid, 27-hydroxyoc
273 function.We tested the effects of high-dose, very-long-chain omega-3 fatty acids on adipose tissue in
275 e activity and approximately 40% decrease in very long chain polyunsaturated fatty acid chain shorten
276 is therefore a key step in the production of very long chain polyunsaturated fatty acid in oil-seed s
277 aturated fatty acids (VLC-FA) and of C28-C38 very long chain polyunsaturated fatty acids (VLC-PUFA),
278 nsiderable beneficial effects of n-3 and n-6 very long chain polyunsaturated fatty acids (VLC-PUFAs),
280 an elongase required for the biosynthesis of very long-chain polyunsaturated fatty acids (VLC-PUFAs).
281 ptor-specific phosphatidylcholine containing very long-chain polyunsaturated fatty acids and severely
282 nthesized in the stroma, then converted into very-long-chain polyunsaturated fatty acids (FAs) at the
283 acid, as well as decreased incorporation of very-long-chain polyunsaturated fatty acids (PUFAs), par
284 mal models clearly indicates that a group of very-long-chain polyunsaturated fatty acids, the n-3 fat
287 enrichment in sterols and sphingolipids with very long chain saturated fatty acids when compared with
289 an oxidase required for the biosynthesis of very-long-chain saturated alpha,omega-bifunctional aliph
290 (DNL) FAs and low n-6 (omega-6) FAs, 2) high very-long-chain saturated FAs, 3) high n-3 (omega-3) FAs
292 id, stearic acid, odd-chain fatty acids, and very-long-chain saturated fatty acids and low concentrat
293 lly expressed in Arabidopsis, GPAT5 produced very-long-chain saturated monoacylglycerols and free fat
295 16:0) are associated with diabetes; however, very-long-chain SFAs (VLSFAs), with 20 or more carbons,
296 llowed by the more challenging separation of very long chain TX-series with a large number (n = 30-70
297 nsation reaction catalyzing the formation of very-long-chain (VLC) (C-28 to C-40) fatty acids, satura
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