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 TmELO2 function to synthesize long chain and 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 utive reactions to preferentially metabolize very long chain fatty acids.

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

43 1/lpcat2 mutant showed increased contents of very-long-chain fatty acids and decreased PUFA in TAG an

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

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

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

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

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

49 The very long chain fatty acids are crucial building blocks

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

71 ,3-enoyl-CoA reduction reactions in long and very long chain fatty acid elongation, respectively.

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

90 Very long-chain fatty acid levels were partially restore

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

116 3-ketoacyl-CoA synthase which is involved in very long chain fatty acid synthesis in vegetative tissu

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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