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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
18 though many patients have been found to have very long-chain acyl-CoA dehydrogenase (VLCAD) deficienc
20 long-chain acyl-CoA dehydrogenase (LCAD) and very long-chain acyl-CoA dehydrogenase revealed that 5-t
21 unsaturated acyl-CoAs are poor substrates of very long-chain acyl-CoA dehydrogenase when compared wit
23 ls suggest that, in contrast to yeast cells, very long-chain acyl-CoA esters are transported into per
24 s been associated with decreased peroxisomal very long-chain acyl-CoA synthetase (VLCS) activity and
25 creased VLCFA beta-oxidation and peroxisomal very long-chain acyl-CoA synthetase (VLCS) activity.
29 002 is a prodrug that requires activation by very long-chain acyl-CoA synthetase-1 (ACSVL1) to modula
35 specifically exhibit down-regulation of the very-long-chain acyl-CoA dehydrogenase (VLCAD) enzyme, w
38 trate specificity, it appears that ACAD9 and very-long-chain acyl-CoA dehydrogenase are unable to com
40 istribution and gene regulation of ACAD9 and very-long-chain acyl-CoA dehydrogenase identify the pres
42 function that is highly homologous to human very-long-chain acyl-CoA dehydrogenase was identified by
43 Gel filtration analysis indicated that, like very-long-chain acyl-CoA dehydrogenase, ACAD-9 is a dime
46 decreased ability of cells to activate (via very-long-chain acyl-coenzyme A synthetase [VLCS]) and s
51 (N-palmitoyl sphingosine) and nonsaturated, very long chain C24:1 ceramide (N-nervonoyl sphingosine)
53 hat confluence caused selective increases in very long chain ceramide C(24:1) (370 +/- 54%) and C(24:
54 bases in blood, accompanied by increases in very long chain ceramide species, and have defective lym
55 d cellular levels of specific long-chain and very long-chain ceramide species relative to vector cont
57 ivering a single dose of radiation, long and very-long-chain ceramide species, and the expression lev
59 Stress increased the amounts of long- and very long-chain ceramides in HEK293 cells, and this was
61 terestingly, elevated levels of only certain very long-chain ceramides were evident in V5-aSMase(S508
63 ication of several endogenous long-chain and very-long-chain ceramides using two nonphysiological odd
65 s in the retina with special emphasis on the very-long-chain-containing saturated (VLC-FA) and polyun
68 d seed coats with a severalfold reduction in very long chain dicarboxylic acid and omega-hydroxy fatt
69 carboxylase, inhibited only elongation into very long chain FAs (>or=20 carbons) but not synthesis o
70 ptor CD36 is required both for the uptake of very long chain FAs (VLCFAs) in cultured cells and the a
72 ed to provide insight into the regulation of very long chain fatty acid (VLCFA) biosynthesis in Brass
73 letion of ALD gene (ABCD1) and the resultant very long chain fatty acid (VLCFA) derangement has drama
76 ese studies reveal roles for Rvs161p and the very long chain fatty acid elongase, Sur4p, in the local
78 ,3-enoyl-CoA reduction reactions in long and very long chain fatty acid elongation, respectively.
79 tly linked to the (omega-1)-hydroxy group of very long chain fatty acid in bradyrhizobial lipid A.
80 Although FATP4 deficiency primarily affected very long chain fatty acid metabolism, mutant fibroblast
82 CoA synthetase that preferentially activates very long chain fatty acid substrates, such as C24:0, to
83 3-ketoacyl-CoA synthase which is involved in very long chain fatty acid synthesis in vegetative tissu
84 r C18:1, suggesting that in vivo, defects in very long chain fatty acid uptake may underlie the skin
85 oblasts, FATP4 is the major enzyme producing very long chain fatty acid-CoA for lipid metabolic pathw
88 cid elongases that catalyze the synthesis of very long chain fatty acids (C24 to C26) required for ce
89 n of VLCFA synthesizing enzymes, elongase of very long chain fatty acids (ELOVLs) (1 and 3) in both c
90 Abnormalities in the transport of saturated very long chain fatty acids (VLCFA; >C18:0) contribute t
91 adrenoleukodystrophy is the accumulation of very long chain fatty acids (VLCFAs) due to impaired per
92 lting in the inability to transport acylated very long chain fatty acids (VLCFAs) into the peroxisome
93 he bubblegum mutant shows elevated levels of very long chain fatty acids (VLCFAs), as seen in the hum
99 receptor drives adults to convert lipids to very long chain fatty acids and hydrocarbons for an anti
100 Rather, dHNF4 directs their conversion to very long chain fatty acids and hydrocarbons, which wate
104 miR-219 and its target ELOVL7 (elongation of very long chain fatty acids protein 7) were identified a
106 oil and fatty acid composition, accumulating Very Long Chain Fatty Acids with industrial applications
107 dehydratases are required for elongation of very long chain fatty acids, and HACD1 has a role in ear
108 lar endothelial cells causes accumulation of very long chain fatty acids, but much later than the imm
111 in the expression of the gene elongation of very long chain fatty acids-like 2, an enzyme needed for
112 ulin-induced gene 2a, Insig2a, elongation of very long chain fatty acids-like 3, Elovl3 and sterol 12
113 A Met299Val variant in the elongation of very long chain fatty acids-like 4 (ELOVL4) gene was sig
126 ption mutant (DeltafoxA) was eliminated on a very long-chain fatty acid (C(22:1)), growth was only pa
127 classes on the stem and leaf, except in the very long-chain fatty acid (VLCFA) class wherein acids l
128 In this study, we investigated the roles of very long-chain fatty acid (VLCFA) synthesis by fatty ac
130 nted the defects in fatty acid transport and very long-chain fatty acid activation associated with a
132 transport protein 4 (FATP4), which each have very long-chain fatty acid acyl-CoA synthetase (VLCFA-AC
133 phingolipid pathway, such as deletion of the very long-chain fatty acid elongase, Sur4, suppress the
135 other acyl-CoA synthetases are necessary for very long-chain fatty acid metabolism in Neuro2a cells.
137 f the ACC and elevation of the ceramides and very long-chain fatty acid syntheses with normal cell-cy
144 defect in synthesis of unsaturated long and very long-chain fatty acids (LCFAs and VLCFAs) and deple
145 Sphingolipids are synthesized de novo from very long-chain fatty acids (VLCFA) and sphingoid long-c
146 bers of this group are capable of activating very long-chain fatty acids (VLCFA), one is a bile acid-
148 re of X-ALD is increased levels of saturated very long-chain fatty acids (VLCFA; carbon chains of 22
150 present study, we found that the contents of very long-chain fatty acids (VLCFAs) in akr2a mutants we
151 (del/del) mice revealed a global decrease in very long-chain fatty acids (VLFAs) (i.e., carbon chain
152 X-ALD is characterized by an accumulation of very long-chain fatty acids and partially impaired perox
153 possibility that intermediate metabolites of very long-chain fatty acids and peroxisomal beta-oxidati
154 p and Elo3p are inviable confirming that the very long-chain fatty acids are essential for cellular f
155 tol-ceramides [IPC, MIPC, and M(IP)(2)C] and very long-chain fatty acids C(24) and C(26) declined sha
156 ssion pattern and ability of BG1 to activate very long-chain fatty acids implicates this enzyme in th
157 icantly inhibited the omega-hydroxylation of very long-chain fatty acids in cultured human keratinocy
158 rely impaired as degradation of unesterified very long-chain fatty acids in X-ALD and is abolished in
159 ferentiation: ELOVL1, encoding elongation of very long-chain fatty acids protein 1, and SLC27A1, enco
162 and Elo3p are required for synthesis of the very long-chain fatty acids, and mutants lacking both El
163 of linoleic acid, odd-chain fatty acids, and very long-chain fatty acids, was associated with lower i
164 l membrane proteins involved in transport of very long-chain fatty acids, which are a unique componen
165 NA library, we have identified elongation of very long-chain fatty acids-like 1 (ELOVL1) and fatty ac
169 long-chain fatty acids and the activation of very long-chain fatty acids; these activities intrinsic
170 ble exceptions, the fatty acid transport and very long-chain fatty acyl-CoA synthetase activities wer
171 ates, the levels of fatty acid accumulation, very long-chain fatty acyl-CoA synthetase activities, an
172 coenzyme A derivatives using long-chain and very-long chain fatty acids, bile acids and bile acid pr
173 ts, led us to discover that BacA affects the very-long-chain fatty acid (27-OHC28:0 and 29-OHC30:0) c
175 es the first and rate-limiting enzyme of the very-long-chain fatty acid (VLCFA) beta-oxidation pathwa
176 and Schefflera elegantissima) contained only very-long-chain fatty acid (VLCFA) derivatives such as a
178 chological, electrophysiological, and plasma very-long-chain fatty acid (VLCFA) measurements were use
179 se complex are required for the synthesis of very-long-chain fatty acid (VLCFA) precursors of cuticul
180 reviously undescribed desaturase activity on very-long-chain fatty acid (VLCFA) substrates and exhibi
181 slation initiation factor 2B (eIF2B) and the very-long-chain fatty acid (VLCFA) synthesis keto-reduct
182 ase type 12 (HSD17B12) as a human hub of the very-long-chain fatty acid (VLCFA) synthesis pathway and
184 and B. abortus are unusually modified with a very-long-chain fatty acid (VLCFA; C > or = 28) and we d
185 ectrometry (LC/MS) method for long-chain and very-long-chain fatty acid analysis and its application
186 not ZS, fibroblasts there was an increase in very-long-chain fatty acid beta-oxidation and plasmaloge
187 X-ALD fibroblasts synergistically increased very-long-chain fatty acid beta-oxidation, indicating th
189 drenoleukodystrophy (X-ALD) is a disorder of very-long-chain fatty acid metabolism, adrenal insuffici
191 results in an increase in phospholipids with very-long-chain fatty acid tails (PL-VLCFAs) that contai
192 distribution of LPS fatty acids, including a very-long-chain fatty acid thought to be unique to the a
194 he PM showed an enrichment of 2-hydroxylated very-long-chain fatty acid-containing GIPCs and polyglyc
195 ision, suggesting that enhanced synthesis of very-long-chain fatty acid/trihydroxy LCB ceramides prom
196 Individuals with ABCD1 mutations accumulate very-long-chain fatty acids (VLCFA) (carbon length >22).
198 X-ALD) is associated with elevated levels of very-long-chain fatty acids (VLCFA; C(>22:0)) that have
203 which exacerbates accumulation of LCFAs and very-long-chain fatty acids (VLCFAs) that mediate lipoto
204 ce blast fungus Magnaporthe oryzae, requires very-long-chain fatty acids (VLCFAs), which act as media
206 des accumulation of peroxisomal educts (like very-long-chain fatty acids [VLCFAs] or branched-chain f
207 flammatory demyelination in the brain, where very-long-chain fatty acids accumulate within phospholip
208 1/lpcat2 mutant showed increased contents of very-long-chain fatty acids and decreased PUFA in TAG an
209 lopmental and environmental requirements for very-long-chain fatty acids and flavonoids, whose synthe
210 ACC (ScACC) is crucial for the production of very-long-chain fatty acids and the maintenance of the n
211 This led to a significant increase in the very-long-chain fatty acids C24 and C26 in the cuticular
213 n GIPCs and sterols and suggested a role for very-long-chain fatty acids in the interdigitation betwe
214 mutations in ABCD1 lead to incorporation of very-long-chain fatty acids into phospholipids, we separ
215 litate the transfer of long-chain as well as very-long-chain fatty acids into the apoplast, depending
218 have shown that larval oenocytes synthesize very-long-chain fatty acids required for tracheal waterp
219 an acyl CoA synthetase activity specific for very-long-chain fatty acids suggesting this protein play
220 (CER6) is necessary for the biosynthesis of very-long-chain fatty acids with chain lengths beyond C(
222 of Elovl proteins that mediate elongation of very-long-chain fatty acids, block or dramatically slow
224 ngation of not only linear but also branched very-long-chain fatty acids, leading to production of th
225 n cause accumulation of unbranched saturated very-long-chain fatty acids, particularly in brain and a
226 pecies with C16 fatty acids rather than with very-long-chain fatty acids, which are more commonly enr
227 ndensing enzyme involved in the synthesis of very-long-chain fatty acids, which are precursors of epi
228 ng patterns of saturated and monounsaturated very-long-chain fatty acids, with the observed pattern c
229 ingle allelic mutations in the elongation of very-long-chain fatty acids-like 4 (ELOVL4), whereas rec
235 We also investigated two distantly related very-long-chain fatty acyl (VLCFA) desaturases from Arab
236 3 (At1g19260)-encoded ceramide synthases use very-long-chain fatty acyl-CoA and trihydroxy LCB substr
237 oxidation chain shortening of long-chain and very-long-chain fatty acyl-coenzyme (CoAs), long-chain d
238 vity of class II ceramide synthases that use very-long-chain fatty acyl-coenzyme A and trihydroxy LCB
239 t jojoba FAO and FADH proteins are active on very-long-chain fatty alcohol and fatty aldehyde substra
240 gest that jojoba FAO and FADH constitute the very-long-chain fatty alcohol oxidation pathway that is
241 Upon germination, WE hydrolysis releases very-long-chain fatty alcohols, which must be oxidized t
242 ax profile (especially on leaves) toward the very-long-chain free fatty acids tetracosanoic acid (C(2
243 pressed in photoreceptor cells and generates very long chain (>/=C28) polyunsaturated fatty acids inc
244 ancer suggest that 1-deoxysphingolipids, the very-long-chain in particular, play a role as molecular
245 istics suggest that it may interact with the very long chain intermediates in mycolic acid biosynthes
246 he other hand, was missing O-antigens of the very long chain length, indicating the Wzz2 protein is r
247 ed with other fatty acids and because of its very long chain length, the effect of dietary behenic ac
248 responsible for the expression of different very long chain lengths despite high sequence homology.
249 out for long chain palmitic acid (C16:0) and very long chain lignoceric acid (C24:0) as well as for A
250 ut for long chain (palmitic acid, C16:0) and very long chain (lignoceric acid, C24:0) fatty acids as
251 suppressor demonstrates that low amounts of very long chain lipids are sufficient for pollen hydrati
253 Mutations in Arabidopsis CER genes eliminate very long chain lipids from the cuticle surface and, in
254 e display massive increases in a rare set of very long chain LPS lipids that have been previously rep
255 he N-acyl chains showed a high occurrence of very-long-chain moieties; phytosphingosine and 4-hydroxy
256 Cuticular wax is a mixture of aliphatic very-long-chain molecules, ranging from 22 to 48 carbons
258 and a dramatic increase in the percentage of very-long-chain monounsaturated fatty acids in the acyl-
259 whether red blood cell (RBC) long-chain and very-long-chain MUFAs are associated with risk of CAD in
264 Higher proportions of certain VLC n-3 and very long-chain n-6 PUFAs in plasma phospholipids at age
265 he effect and the mechanism of action of the very-long-chain n-3 (omega-3) polyunsaturated fatty acid
267 feasibility for CEC-ESI-MS analysis of these very long chain nonionic surfactants for the first time.
268 in place of the proximal glucosamine, and a very long chain omega-1 hydroxy fatty acid, 27-hydroxyoc
270 function.We tested the effects of high-dose, very-long-chain omega-3 fatty acids on adipose tissue in
272 e activity and approximately 40% decrease in very long chain polyunsaturated fatty acid chain shorten
273 is therefore a key step in the production of very long chain polyunsaturated fatty acid in oil-seed s
274 aturated fatty acids (VLC-FA) and of C28-C38 very long chain polyunsaturated fatty acids (VLC-PUFA),
275 nsiderable beneficial effects of n-3 and n-6 very long chain polyunsaturated fatty acids (VLC-PUFAs),
277 duction of phospholipids containing 22:6 and very long-chain polyunsaturated fatty acids (VLC-PUFAs)
278 an elongase required for the biosynthesis of very long-chain polyunsaturated fatty acids (VLC-PUFAs).
279 ptor-specific phosphatidylcholine containing very long-chain polyunsaturated fatty acids and severely
280 nthesized in the stroma, then converted into very-long-chain polyunsaturated fatty acids (FAs) at the
281 acid, as well as decreased incorporation of very-long-chain polyunsaturated fatty acids (PUFAs), par
282 mal models clearly indicates that a group of very-long-chain polyunsaturated fatty acids, the n-3 fat
285 enrichment in sterols and sphingolipids with very long chain saturated fatty acids when compared with
286 an oxidase required for the biosynthesis of very-long-chain saturated alpha,omega-bifunctional aliph
287 (DNL) FAs and low n-6 (omega-6) FAs, 2) high very-long-chain saturated FAs, 3) high n-3 (omega-3) FAs
289 id, stearic acid, odd-chain fatty acids, and very-long-chain saturated fatty acids and low concentrat
290 lly expressed in Arabidopsis, GPAT5 produced very-long-chain saturated monoacylglycerols and free fat
293 hat higher circulating concentrations of the very-long-chain SFAs (VLSFAs) arachidic acid (20:0), beh
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