ライフサイエンスコーパス: very-long-chain fatty acid

1 mutations in the ELOVL4 gene (elongation of very-long-chain fatty acids).

2 s7p is required for the hydroxylation of the very long chain fatty acid.

3 the condensation of a long chain base with a very long chain fatty acid.

4 nounsaturated fatty acids and a reduction in very long chain fatty acids.

5 gation of a second stearate molecule to form very long chain fatty acids.

6 active on long chain fatty acids relative to very long chain fatty acids.

7 saturases, enzymes involved in elongation of very long chain fatty acids.

8 roteins that function in the biosynthesis of very long chain fatty acids.

9 kodystrophy protein, with an accumulation of very long chain fatty acids.

10 TmELO2 function to synthesize long chain and very long chain fatty acids.

11 utive reactions to preferentially metabolize very long chain fatty acids.

12 and ELO3 produce defects in the formation of very long chain fatty acids.

13 in the ABCD1 gene leading to accumulation of very long chain fatty acids.

14 haracterized by the abnormal accumulation of very long chain fatty acids.

15 activity, with an apparent preference toward very long chain fatty acids.

16 ormalities in these cells are not limited to very long chain fatty acids.

17 nd C24:0 as substrates), and accumulation of very long-chain fatty acids.

18 xogenous fatty acids or in the activation of very long-chain fatty acids.

19 t-chain and severely restricted on long- and very long-chain fatty acids.

20 methyl group addition occurs was a family of very long-chain fatty acids.

21 er dextrose or oleic acid medium accumulated very long-chain fatty acids.

22 hase--an enzyme involved in the synthesis of very-long-chain fatty acids.

23 file, with an increase in triglycerides with very-long-chain fatty acids.

24 showed that AtLtpI-4 protein can bind these very-long-chain fatty acids.

25 tent, with some molecular species containing very-long-chain fatty acids.

26 ber of genes involved in the biosynthesis of very-long-chain fatty acids.

27 long-chain fatty acids and the activation of very-long-chain fatty acids.

28 etric screen for inhibitors of elongation of very long chain fatty acid 1 (ELOVL1) enzyme.

29 ach based on the inhibition of elongation of very long chain fatty acid 1 enzyme (ELOVL1), we explore

30 ts, led us to discover that BacA affects the very-long-chain fatty acid (27-OHC28:0 and 29-OHC30:0) c

31 Recently, elongase of very long chain fatty acids-3 (ELOVL3) was demonstrated

32 Elongation of very long chain fatty acids 4 (ELOVL4) is a novel member

33 ed in exon six of a gene named Elongation of very long chain fatty acids 4 (ELOVL4).

34 ed by mutations in the ELOVL4 (Elongation of very long chain fatty acids 4) gene.

35 Elongation of very long chain fatty acids-4 (ELOVL4) has been identifi

36 Mutations in elongation of very long-chain fatty acid-4 (ELOVL4) are associated wit

37 tty acid desaturase 2 (FADS2), elongation of very long-chain fatty acid 5 (ELOVL5) and long-chain acy

38 flammatory demyelination in the brain, where very-long-chain fatty acids accumulate within phospholip

39 ecrease in peroxisomal VLCS activity and the very long-chain fatty acid accumulation in the yeast FAT

40 nted the defects in fatty acid transport and very long-chain fatty acid activation associated with a

41 mBG1 appears to play a minor role in very long-chain fatty acid activation in these cells, in

42 transport protein 4 (FATP4), which each have very long-chain fatty acid acyl-CoA synthetase (VLCFA-AC

43 Major cuticular wax compounds, such as very long-chain fatty acids, alcohols, alkanes, and keto

44 ectrometry (LC/MS) method for long-chain and very-long-chain fatty acid analysis and its application

45 nvolved in the peroxisomal beta-oxidation of very long chain fatty acids and are associated with defe

46 receptor drives adults to convert lipids to very long chain fatty acids and hydrocarbons for an anti

47 Rather, dHNF4 directs their conversion to very long chain fatty acids and hydrocarbons, which wate

48 contain unique lipids such as mycolic acids, very long chain fatty acids and multimethyl-branched fat

49 Cuticular waxes are complex mixtures of very long chain fatty acids and their derivatives that c

50 X-ALD is characterized by an accumulation of very long-chain fatty acids and partially impaired perox

51 possibility that intermediate metabolites of very long-chain fatty acids and peroxisomal beta-oxidati

52 1/lpcat2 mutant showed increased contents of very-long-chain fatty acids and decreased PUFA in TAG an

53 lopmental and environmental requirements for very-long-chain fatty acids and flavonoids, whose synthe

54 ACC (ScACC) is crucial for the production of very-long-chain fatty acids and the maintenance of the n

55 dehydratases are required for elongation of very long chain fatty acids, and HACD1 has a role in ear

56 golipids, substantial levels of hydroxylated very long chain fatty acids, and the full complement of

57 function include ceramides, cholesterol, and very long chain fatty acids, and their alteration causes

58 and Elo3p are required for synthesis of the very long-chain fatty acids, and mutants lacking both El

59 The very long chain fatty acids are crucial building blocks

60 p and Elo3p are inviable confirming that the very long-chain fatty acids are essential for cellular f

61 Utilizing the plasma very long chain fatty acid assay, supplemented by mutati

62 , which is responsible for the elongation of very long-chain fatty acids (at least 26 carbons).

63 not ZS, fibroblasts there was an increase in very-long-chain fatty acid beta-oxidation and plasmaloge

64 X-ALD fibroblasts synergistically increased very-long-chain fatty acid beta-oxidation, indicating th

65 rates, including branched-chain fatty acids, very long-chain fatty acids, bile salt intermediates, an

66 coenzyme A derivatives using long-chain and very-long chain fatty acids, bile acids and bile acid pr

67 of Elovl proteins that mediate elongation of very-long-chain fatty acids, block or dramatically slow

68 is canonically involved in the synthesis of very-long-chain fatty acids but appears to promote nonap

69 lar endothelial cells causes accumulation of very long chain fatty acids, but much later than the imm

70 tol-ceramides [IPC, MIPC, and M(IP)(2)C] and very long-chain fatty acids C(24) and C(26) declined sha

71 ption mutant (DeltafoxA) was eliminated on a very long-chain fatty acid (C(22:1)), growth was only pa

72 tachyzoites synthesized a range of long and very long chain fatty acids (C14:0-26:1).

73 This led to a significant increase in the very-long-chain fatty acids C24 and C26 in the cuticular

74 cid elongases that catalyze the synthesis of very long chain fatty acids (C24 to C26) required for ce

75 ese cells are devoid of ABCD1 and accumulate very long-chain fatty acids (C26:0 and C26:1).

76 In a yeast strain engineered to produce very-long-chain fatty acids, CER1-LIKE1 interacted with

77 oblasts, FATP4 is the major enzyme producing very long chain fatty acid-CoA for lipid metabolic pathw

78 lasmalogen concentrations, and a decrease in very-long-chain fatty acid concentrations.

79 he PM showed an enrichment of 2-hydroxylated very-long-chain fatty acid-containing GIPCs and polyglyc

80 CROT is a peroxisomal enzyme catalyzing very long-chain fatty acids conversion to medium chain f

81 Degradation of very long chain fatty acids decreased by 47% and resulte

82 metabolic functions, including oxidation of very long-chain fatty acids, degradation of D-amino acid

83 ember 16, glucose 6-phosphate dehydrogenase, very long chain fatty acid elongase, and phospholipase A

84 ese studies reveal roles for Rvs161p and the very long chain fatty acid elongase, Sur4p, in the local

85 phingolipid pathway, such as deletion of the very long-chain fatty acid elongase, Sur4, suppress the

86 sequence identity with previously identified very long chain fatty acid elongases.

87 ,3-enoyl-CoA reduction reactions in long and very long chain fatty acid elongation, respectively.

88 The elongases of very long chain fatty acid (ELOVL or ELO) are essential

89 s of TMEM120A resemble those of elongase for very long-chain fatty acids (ELOVL) despite the low sequ

90 was negatively associated with elongation of very long-chain fatty acids (ELOVL6; C(16:0)->C(18:0)) e

91 n of VLCFA synthesizing enzymes, elongase of very long chain fatty acids (ELOVLs) (1 and 3) in both c

92 Here we show that the very long-chain fatty acid erucic acid (EA) triggers acy

93 We showed that NLMs lost saturated very-long-chain fatty acid (FA; C24:0) SM in cancer cell

94 tivity that is essential for normal cellular very long-chain fatty acid homeostasis.

95 n precursor, suggesting that it functions in very long chain fatty acid hydroxylation using an altern

96 ssion pattern and ability of BG1 to activate very long-chain fatty acids implicates this enzyme in th

97 tly linked to the (omega-1)-hydroxy group of very long chain fatty acid in bradyrhizobial lipid A.

98 he expression of ACOX protein and accumulate very long chain fatty acids in blood.

99 mical parameters, including accumulations of very long chain fatty acids in plasma and deficient eryt

100 icantly inhibited the omega-hydroxylation of very long-chain fatty acids in cultured human keratinocy

101 rely impaired as degradation of unesterified very long-chain fatty acids in X-ALD and is abolished in

102 synthetase [VLCS]) and subsequently degrade very-long-chain fatty acids in peroxisomes.

103 n GIPCs and sterols and suggested a role for very-long-chain fatty acids in the interdigitation betwe

104 ed levels of and increased beta-oxidation of very-long-chain fatty acids; increased expression of the

105 primary rodent neurons and astrocytes after very long chain fatty acid-induced toxicity simulating X

106 mutations in ABCD1 lead to incorporation of very-long-chain fatty acids into phospholipids, we separ

107 litate the transfer of long-chain as well as very-long-chain fatty acids into the apoplast, depending

108 defect in synthesis of unsaturated long and very long-chain fatty acids (LCFAs and VLCFAs) and deple

109 ngation of not only linear but also branched very-long-chain fatty acids, leading to production of th

110 Very long-chain fatty acid levels were partially restore

111 its production of a substantial reduction of very-long-chain fatty acid levels in the brain and adren

112 ELOVL2 (elongation of very long chain fatty acids-like 2) encodes a transmembr

113 in the expression of the gene elongation of very long chain fatty acids-like 2, an enzyme needed for

114 ulin-induced gene 2a, Insig2a, elongation of very long chain fatty acids-like 3, Elovl3 and sterol 12

115 A Met299Val variant in the elongation of very long chain fatty acids-like 4 (ELOVL4) gene was sig

116 NA library, we have identified elongation of very long-chain fatty acids-like 1 (ELOVL1) and fatty ac

117 ingle allelic mutations in the elongation of very-long-chain fatty acids-like 4 (ELOVL4), whereas rec

118 We found very long chain fatty acids, medium chain acylcarnitines

119 Although FATP4 deficiency primarily affected very long chain fatty acid metabolism, mutant fibroblast

120 other acyl-CoA synthetases are necessary for very long-chain fatty acid metabolism in Neuro2a cells.

121 drenoleukodystrophy (X-ALD) is a disorder of very-long-chain fatty acid metabolism, adrenal insuffici

122 ALD) is a peroxisomal disorder with impaired very-long-chain fatty acid metabolism.

123 membrane, the so-called mycomembrane made of very-long chain fatty acids, named mycolic acids.

124 S), the enzyme responsible for production of very long chain fatty acids of plant seed oils.

125 Our findings implicate very-long-chain fatty acids or their derivative complex

126 ein, required for the efficient transport of very-long-chain fatty acids out of the cytoplasm.

127 etabolic pathways, ether lipid synthesis and very long chain fatty acid oxidation.

128 , were linked with reduced beta-oxidation of very long chain fatty acids (p = 0.004).

129 n cause accumulation of unbranched saturated very-long-chain fatty acids, particularly in brain and a

130 Here, we show that ELOngation of Very Long chain fatty acids protein 4 (ELOVL4), a rate-l

131 miR-219 and its target ELOVL7 (elongation of very long chain fatty acids protein 7) were identified a

132 ferentiation: ELOVL1, encoding elongation of very long-chain fatty acids protein 1, and SLC27A1, enco

133 Mycolic acids are very long-chain fatty acids representing essential compo

134 have shown that larval oenocytes synthesize very-long-chain fatty acids required for tracheal waterp

135 Analysis of sphingolipid-derived very long chain fatty acids revealed an approximately 40

136 Reduction of very-long-chain fatty acid sphingolipid levels leads in

137 CoA synthetase that preferentially activates very long chain fatty acid substrates, such as C24:0, to

138 and is capable of activating both long- and very long-chain fatty acid substrates.

139 an acyl CoA synthetase activity specific for very-long-chain fatty acids suggesting this protein play

140 f the ACC and elevation of the ceramides and very long-chain fatty acid syntheses with normal cell-cy

141 3-ketoacyl-CoA synthase which is involved in very long chain fatty acid synthesis in vegetative tissu

142 synthase that catalyzes the initial step of very long-chain fatty acid synthesis.

143 s was most prominent for enzymes involved in very long-chain fatty acid synthesis.

144 results in an increase in phospholipids with very-long-chain fatty acid tails (PL-VLCFAs) that contai

145 longation systems that produce the 26-carbon very long chain fatty acids that are precursors for cera

146 long-chain fatty acids and the activation of very long-chain fatty acids; these activities intrinsic

147 distribution of LPS fatty acids, including a very-long-chain fatty acid thought to be unique to the a

148 ells to be more efficiently utilizing stored very long-chain fatty acids to survive nutrient depletio

149 n the ABCD1 gene, producing mutations in the very long chain fatty acid transporter, ALD protein.

150 ision, suggesting that enhanced synthesis of very-long-chain fatty acid/trihydroxy LCB ceramides prom

151 r C18:1, suggesting that in vivo, defects in very long chain fatty acid uptake may underlie the skin

152 smembrane proteins that facilitate long- and very long-chain fatty acid uptake.

153 amily of six FATPs that facilitate long- and very long-chain fatty acid uptake.

154 amily of six FATPs that facilitate long- and very-long-chain fatty acid uptake.

155 lying SCA34 pathology.SIGNIFICANCE STATEMENT Very long chain-fatty acids (VLC-FA) are an understudied

156 ty acids-4 (ELOVL4) mediates biosynthesis of very long chain-fatty acids (VLC-FA; >=28 carbons).

157 isorder characterized by reduced peroxisomal very long chain fatty acid (VLCFA) beta-oxidation.

158 ed to provide insight into the regulation of very long chain fatty acid (VLCFA) biosynthesis in Brass

159 letion of ALD gene (ABCD1) and the resultant very long chain fatty acid (VLCFA) derangement has drama

160 roxisomal transmembrane protein required for very long chain fatty acid (VLCFA) metabolism.

161 nzyme A oxidase 1 (ACOX1), which catabolizes very long chain fatty acids (VLCFA), increases in the co

162 Abnormalities in the transport of saturated very long chain fatty acids (VLCFA; >C18:0) contribute t

163 classes on the stem and leaf, except in the very long-chain fatty acid (VLCFA) class wherein acids l

164 In this study, we investigated the roles of very long-chain fatty acid (VLCFA) synthesis by fatty ac

165 the elongase system of enzymes required for very long-chain fatty acid (VLCFA) synthesis.

166 aused by mutations in ABCD1, the peroxisomal very long-chain fatty acid (VLCFA) transporter.

167 Sphingolipids are synthesized de novo from very long-chain fatty acids (VLCFA) and sphingoid long-c

168 bers of this group are capable of activating very long-chain fatty acids (VLCFA), one is a bile acid-

169 ve disorder characterized by accumulation of very long-chain fatty acids (VLCFA).

170 re of X-ALD is increased levels of saturated very long-chain fatty acids (VLCFA; carbon chains of 22

171 es the first and rate-limiting enzyme of the very-long-chain fatty acid (VLCFA) beta-oxidation pathwa

172 and Schefflera elegantissima) contained only very-long-chain fatty acid (VLCFA) derivatives such as a

173 Notably, an unusual very-long-chain fatty acid (VLCFA) is found in the lipid

174 chological, electrophysiological, and plasma very-long-chain fatty acid (VLCFA) measurements were use

175 se complex are required for the synthesis of very-long-chain fatty acid (VLCFA) precursors of cuticul

176 reviously undescribed desaturase activity on very-long-chain fatty acid (VLCFA) substrates and exhibi

177 slation initiation factor 2B (eIF2B) and the very-long-chain fatty acid (VLCFA) synthesis keto-reduct

178 ase type 12 (HSD17B12) as a human hub of the very-long-chain fatty acid (VLCFA) synthesis pathway and

179 CS2), which catalyze two successive steps in very-long-chain fatty acid (VLCFA) synthesis.

180 and B. abortus are unusually modified with a very-long-chain fatty acid (VLCFA; C > or = 28) and we d

181 Individuals with ABCD1 mutations accumulate very-long-chain fatty acids (VLCFA) (carbon length >22).

182 Very-long-chain fatty acids (VLCFA) and branched-chain f

183 X-ALD) is associated with elevated levels of very-long-chain fatty acids (VLCFA; C(>22:0)) that have

184 mal disorder with impaired beta-oxidation of very long chain fatty acids (VLCFAs) and reduced functio

185 Very long chain fatty acids (VLCFAs) are precursors to s

186 Accumulation of very long chain fatty acids (VLCFAs) due to defects in A

187 adrenoleukodystrophy is the accumulation of very long chain fatty acids (VLCFAs) due to impaired per

188 lting in the inability to transport acylated very long chain fatty acids (VLCFAs) into the peroxisome

189 he bubblegum mutant shows elevated levels of very long chain fatty acids (VLCFAs), as seen in the hum

190 The assay of plasma very long chain fatty acids (VLCFAs), developed in our l

191 lti-component fatty acid elongase to produce very long chain fatty acids (VLCFAs), which are building

192 id elongase required for the biosynthesis of very long chain fatty acids (VLCFAs).

193 ngation pathway responsible for formation of very long chain fatty acids (VLCFAs, or fatty acids with

194 eroxisomal matrix protein import, leading to very long-chain fatty acids (VLCFAs) accumulation.

195 orating in lipid metabolism, for example, of very long-chain fatty acids (VLCFAs) and plasmalogens, t

196 Very long-chain fatty acids (VLCFAs) are essential lipid

197 present study, we found that the contents of very long-chain fatty acids (VLCFAs) in akr2a mutants we

198 Here, we show that sphingolipids containing very long-chain fatty acids (VLCFAs) promote homotypic v

199 ntly reduced the proportion of corresponding very long-chain fatty acids (VLCFAs) species (C22-28) co

200 Long-chain and very-long-chain fatty acids (VLCFAs) are also metabolize

201 Among lipid species, the very-long-chain fatty acids (VLCFAs) are relatively rare

202 The extension of very-long-chain fatty acids (VLCFAs) for the synthesis o

203 TSC13 is required for the biosynthesis of very-long-chain fatty acids (VLCFAs) in yeast.

204 which exacerbates accumulation of LCFAs and very-long-chain fatty acids (VLCFAs) that mediate lipoto

205 ALD) is caused by loss of the transporter of very-long-chain fatty acids (VLCFAs), ABCD1.

206 ce blast fungus Magnaporthe oryzae, requires very-long-chain fatty acids (VLCFAs), which act as media

207 , including fatty acid elongation to produce very-long-chain fatty acids (VLCFAs).

208 des accumulation of peroxisomal educts (like very-long-chain fatty acids [VLCFAs] or branched-chain f

209 (del/del) mice revealed a global decrease in very long-chain fatty acids (VLFAs) (i.e., carbon chain

210 In contrast, synthesis of very long chain fatty acids was primarily dependent on a

211 of linoleic acid, odd-chain fatty acids, and very long-chain fatty acids, was associated with lower i

212 Very long-chain fatty acids, which accumulate in tissues

213 l membrane proteins involved in transport of very long-chain fatty acids, which are a unique componen

214 pecies with C16 fatty acids rather than with very-long-chain fatty acids, which are more commonly enr

215 ndensing enzyme involved in the synthesis of very-long-chain fatty acids, which are precursors of epi

216 oil and fatty acid composition, accumulating Very Long Chain Fatty Acids with industrial applications

217 (CER6) is necessary for the biosynthesis of very-long-chain fatty acids with chain lengths beyond C(

218 ng patterns of saturated and monounsaturated very-long-chain fatty acids, with the observed pattern c