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[PMID]:27116985
[Au] Autor:Meng R; Yang Z; Wang HL; Han YW; Wang YL; Yu H
[Ad] Endereço:Department of Obstetrics and Gynecology, Peking University Third Hospital, Beijing 100191, China.
[Ti] Título:[Variation of long-chain 3-hydroxyacyl CoA dehydrogenase DNA methylated modification and correlation with gene mRNA expression of early-onset preeclampsia, HELLP syndrome and antiphospholipid syndrome in trophoblast cells of placenta].
[So] Source:Zhonghua Fu Chan Ke Za Zhi;51(4):270-8, 2016 Apr 25.
[Is] ISSN:0529-567X
[Cp] País de publicação:China
[La] Idioma:chi
[Ab] Resumo:OBJECTIVE: By detecting the DNA methylation and gene expression of long-chain 3-hydroxyacyl CoA dehydrogenase(LCHAD)in trophoblast cells, analyze the correlation of DNA methylation and gene expression in early-onset preeclampsia(EPE), hemolysis, elevated liver enzymes, and low platelets(HELLP)syndrome and antiphospholipid syndrome(APS), to investigate the molecular basis of long-chain fatty acid oxidation changes in different preeclampsia and pathological pregnancy. METHODS: Primary human cytotrophoblast cells and HTR8/Svneo cells were treated with serum from patients with EPE(14 cases), HELLP(12 cases), APS(14 cases), and normal pregnant women(NP, 14 cases). The methylation level of LCHAD gene promoter region through the MassARRAY platform and mRNA expression level by real-time fluorescent quantitative PCR technique were conducted. RESULTS: (1)Cytosine-phosphate-guanine(CpG)sites in human LCHAD DNA promoter region: CpG sites were detected in the range of 558 bp before LCHAD gene transcription start site, the detected CpG sites were 11 sites including 8 single sites and 3 complex sites. The position of these sites were at-984,-960,-899,-853,-811,-796,-774,-727,-615,-595,-579 respectively.(2)The sites of-899,-853,-615 and-595 showed increased methylation level in EPE and HELLP groups. The methylation level at-899,-853 and-615 sites in EPE and HELLP groups were significantly higher than those in NP group(P<0.01). The methylation level at-853 site was higher in EPE group than that in HELLP group(P<0.05). The-595 site showed the unmethylated in EPE, HELLP and APS groups. There were significantly difference between the 3 groups and EPE group(P<0.01).(3)The gene expression of LCHAD mRNA in EPE(0.048±0.005), HELLP(0.045±0.006)and APS(0.044±0.004)groups were significantly lower than NP group(0.076±0.009; P<0.01).(4)The correlation of methylation level and gene expression in all groups: the methylation level at-899,-853,-727,-615 and-579 sites were negatively correlated with gene mRNA expression in EPE group(P<0.05). The methylation level at-899,-853 and-615 sites were negatively correlated with gene mRNA expression in HELLP group(P< 0.05). CONCLUSIONS: The variation of LCHAD DNA methylation of trophoblast cells are found among EPE, HELLP syndrome and APS. The different correlation of LCHAD DNA methylation and gene expression are different in pathological groups. LCHAD DNA methylation of EPE and HELLP syndrome were significantly increased and negatively correlated with LCHAD gene mRNA expression. These results further revealed the molecular basis of long-chain fatty acid oxidation in different preeclampsia and pathological pregnancy.
[Mh] Termos MeSH primário: Síndrome Antifosfolipídica/genética
Metilação de DNA/genética
Síndrome HELLP/genética
3-Hidroxiacil-CoA Desidrogenase de Cadeia Longa/metabolismo
Pré-Eclâmpsia/genética
Pré-Eclâmpsia/metabolismo
[Mh] Termos MeSH secundário: 3-Hidroxiacil-CoA Desidrogenases/genética
Cardiomiopatias
DNA
Ácidos Graxos
Feminino
Expressão Gênica
Seres Humanos
Erros Inatos do Metabolismo Lipídico
Miopatias Mitocondriais
Proteína Mitocondrial Trifuncional/deficiência
Doenças do Sistema Nervoso
Oxirredução
Placenta/metabolismo
Gravidez
RNA Mensageiro/genética
Rabdomiólise
Trofoblastos
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Fatty Acids); 0 (RNA, Messenger); 9007-49-2 (DNA); EC 1.1.1.- (3-Hydroxyacyl CoA Dehydrogenases); EC 1.1.1.211 (Long-Chain-3-Hydroxyacyl-CoA Dehydrogenase); EC 2.3.1.16 (Mitochondrial Trifunctional Protein)
[Em] Mês de entrada:1701
[Cu] Atualização por classe:170111
[Lr] Data última revisão:
170111
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:160428
[St] Status:MEDLINE
[do] DOI:10.3760/cma.j.issn.0529-567X.2016.04.006


  2 / 97 MEDLINE  
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[PMID]:26711077
[Au] Autor:Meng R; Yang Z; Wang H; Wang Y; Li F; Han Y
[Ad] Endereço:Department of Obstetrics and Gynecology, Peking University Third Hospital, Beijing 100191, China.
[Ti] Título:[Study on the methylation of LCHAD gene promoter region in mitochondria of trophoblast cells incubated with long-chain fatty acids].
[So] Source:Zhonghua Yi Xue Za Zhi;95(29):2387-92, 2015 Aug 04.
[Is] ISSN:0376-2491
[Cp] País de publicação:China
[La] Idioma:chi
[Ab] Resumo:OBJECTIVE: To explore the methylation level of long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) gene promoter region in mitochondria of trophoblasts incubated with long-chain fatty acids and the time-effect of methylation modification. METHODS: Primary human trophoblast cells and HTR8/Svneo cells were incubated with free fatty acids of various lengths. Long-chain free fatty acids (LC-FFA) was experimental group, short-chain fatty acids (SC-FFA) and medium-chain fatty acids (MC-FFA) were control groups, and blank control was without free fatty acid. Collecting cells and extract DNA at 24, 48 and 72 h incubation respectively. Predicted CpG island location access to 2 000 bp DNA sequences upstream of the transcription start site of LCHAD gene. We designed methylation detection sites and primer originally. Methylation of CpG sites in LCHAD gene promoter region were detected by MassARRAY and analyzed statistically. RESULTS: (1) We detected methylation of 65% (11/17) CpG sites, including 8 single sites and 3 composite sites, in ampliconic sequences. These CpG sites were at positions: -984, -960, -899, -853, -811, -796, -774, -727, -615, -595, -579, respectively. In different groups methylation level and changes of every site showed differences with the most significant changes at -899 site. (2) Methylation of CpG island in LCHAD gene promoter region: â‘  Methylation of CpG island in LC-FFA and MC-FFA groups showed rising trend with the time: Methylation level of LC-FFA group at 72 h (0.55±0.08) was significantly higher than that of 48 h (0.35±0.12) and 24 h (0.31±0.04) (P<0.05). Methylation level of MC-FFA group at 72 h (0.44±0.05) was significantly higher than that of 24 h (0.31±0.04) (P<0.05). â‘¡ Methylation of CpG island in LCHAD gene promoter region in different groups at different times: Methylation level of LC-FFA group at 72 h was significantly higher than that of the other two groups at 72, 48 and 24 h (P<0.05). (3) Methylation of 11 CpG sites in LCHAD gene promoter region in different groups at 72 h: Methylation level of -899 site in LC-FFA and MC-FFA groups were significantly higher than that of other sites (P<0.05). (4) Methylation level of -899 site in LCHAD gene promoter region in LC-FFA group showed rising trend with the time: (72 h (0.34±0.15), 48 h (0.14±0.05) and 24 h (0.10±0.02), P<0.05). Methylation level of LC-FFA group at 72 h was significantly higher than that of the other two groups at 72, 48 and 24 h (P<0.05). CONCLUSIONS: Methylation modification effect on LCHAD gene promoter region in trophoblast cells incubated with long-chain fatty acids is more significant than with medium-chain and short-chain fatty acids and shows obvious time-effect as incubation time prolonged. The changes at -899 site dominate the degree of methylation in LCHAD gene promoter region.
[Mh] Termos MeSH primário: Mitocôndrias
Regiões Promotoras Genéticas
Trofoblastos
[Mh] Termos MeSH secundário: Células Cultivadas
Metilação de DNA
Ácidos Graxos
Seres Humanos
3-Hidroxiacil-CoA Desidrogenase de Cadeia Longa
Oxirredução
[Pt] Tipo de publicação:ENGLISH ABSTRACT; JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Fatty Acids); EC 1.1.1.211 (Long-Chain-3-Hydroxyacyl-CoA Dehydrogenase)
[Em] Mês de entrada:1602
[Cu] Atualização por classe:151229
[Lr] Data última revisão:
151229
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:151230
[St] Status:MEDLINE


  3 / 97 MEDLINE  
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[PMID]:26675572
[Au] Autor:Han Y; Yang Z; Ding X; Yu H; Yi Y
[Ad] Endereço:Department of Obstetrics and Gynecology, Peking University Third Hospital, Beijing 100191, China.
[Ti] Título:[Variation of long-chain 3-hydroxyacyl-CoA dehydrogenase DNA methylation in placenta of different preeclampsia-like mouse models].
[So] Source:Zhonghua Fu Chan Ke Za Zhi;50(10):740-6, 2015 Oct.
[Is] ISSN:0529-567X
[Cp] País de publicação:China
[La] Idioma:chi
[Ab] Resumo:OBJECTIVE: By detecting the variation of long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) DNA methylation in preeclampsia-like mouse models generated by different ways, to explore the roles of multifactor and multiple pathways in preeclampsia pathogenesis on molecular basis. METHODS: Established preeclampsia-like mouse models in different ways and divided into groups as follows: (1) Nw-nitro-L-arginine-methyl ester (L-NAME) group: wild-type pregnant mouse received subcutaneous injection of L-NAME; (2) lipopolysaccharide (LPS) group: wild-type pregnant mouse received intraperitoneal injection of LPS; (3) apolipoprotein C-III (ApoC3) group: ApoC3 transgenic pregnant mouse with dysregulated lipid metabolism received subcutaneous injection of L-NAME; (4) ß2 glycoprotein I (ß-2GPI) group: wild-type pregnant mouse received subcutaneous injection of ß-2GPI. According to the first injection time (on day 3, 11, 16 respectively), the L-NAME, LPS and ApoC3 groups were further subdivided into: pre-implantation (PI) experimental stage, early gestation (EG) experimental stage, and late gestation (LG) experimental stage. ß-2GPI group was only injected before implantation. LCHAD gene methylation levels in placental were detected in different experimental stage. Normal saline control groups were set within wild-type and ApoC3 transgenic pregnant mice simultaneously. RESULTS: (1) CG sites in LCHAD DNA: 45 CG sites were detected in the range of 728 bp before LCHAD gene transcription start site, the 5, 12, 13, 14, 15, 16, 19, 24, 25, 27, 28, 29, 30, 31, 32, 34, 35, 43 CG sites were complex sites which contained two or more CG sequences, others were single site which contained one CG sequence. The 3, 5, 6, 11, 13, 14, 18, 28 sites in L-NAME, LPS, ApoC3 and ß-2GPI groups showed different high levels of methylation; the 16, 25, 31, 42, 44 sites showed different low levels of methylation; other 32 sites were unmethylated. (2) Comparison of LCHAD gene methylation between different groups: the methylation levels of LCAHD gene at 3, 11, 13, 14, 18 sites in L-NAME, LPS, ApoC3 and ß-2GPI groups were significantly higher than those in the normal saline control group (P < 0.05); and the methylation levels of 42, 44 sites in these groups were significantly lower than those in the normal saline control group (P < 0.05). (3) Methylation of LCHAD gene at the same site between different experimental stages: â‘  The 3, 11, 18 sites of EG experimental stage was significantly lower than PI and LG experimental stage in L-NAME group (P < 0.05); the 3, 11, 18 sites of PI experimental stage was significantly lower than EG and LG experimental stage in LPS group (P < 0.05); these sites of PI experimental stage was significantly higher than EG and LG experimental stages in ApoC3 group (P < 0.05). â‘¡ The methylation of site 5 in L-NAME and LPS groups were significantly higher than that of the normal saline control group (P < 0.05), and the LG experimental stages were significantly higher than other stages, but in ApoC3 group, only PI and EG stages were significantly higher than the normal saline control group (P < 0.05). â‘¢ At site 6 in L-NAME group which showed high methylation level was significantly higher than the same site in other groups which showed low methylation level (P < 0.05). â‘£ At 13, 14 sites, earlier preeclampsia onset caused a lower methylation level in L-NAME group, but PI experimental stage was significantly higher than EG and LG experimental stages in LPS group (P < 0.05), EG experimental stage was significantly higher than PI and LG experimental stages in ApoC3 group (P < 0.05). ⑤ At site 28, earlier preeclampsia onset caused a higher methylation level in L-NAME group, but PI experimental stage was significantly lower than EG and LG experimental stages in LPS group (P < 0.05), EG experimental stage was significantly higher than PI and LG experimental stages in ApoC3 group (P < 0.05). â‘¥ The 16, 25, 31 sites in ApoC3 group were significantly higher than other groups (P < 0.05). ⑦ At site 42 in ß-2GPI group was unmethylated, but it in other groups showed low methylation level, the methylation level of site 42 in ß-2GPI group was significantly lower than that in other groups (P < 0.05). CONCLUSIONS: The methylation of 6 and 42 CG sites may be related to LCHAD gene expression in placenta of L-NAME and ß-2GPI induced preeclampsia-like models respectively; LCHAD gene expression and DNA methylation may not have obvious correlation in LPS and ApoC3 induced preeclampsia-like models. Differences exist in LCHAD DNA methylation in preeclampsia-like models generated by different ways, revealed a molecular basis to expand our understanding of the multi-factorial pathogenesis of preeclampsia.
[Mh] Termos MeSH primário: 3-Hidroxiacil-CoA Desidrogenases/deficiência
Arginina/análogos & derivados
Cardiomiopatias/genética
Metilação de DNA/genética
Erros Inatos do Metabolismo Lipídico/genética
3-Hidroxiacil-CoA Desidrogenase de Cadeia Longa/metabolismo
Miopatias Mitocondriais/genética
Doenças do Sistema Nervoso/genética
Pré-Eclâmpsia/metabolismo
Rabdomiólise/genética
[Mh] Termos MeSH secundário: 3-Hidroxiacil-CoA Desidrogenases/genética
3-Hidroxiacil-CoA Desidrogenase
Animais
Arginina/genética
Modelos Animais de Doenças
Ácidos Graxos
Feminino
Seres Humanos
Metabolismo dos Lipídeos
Camundongos
Proteína Mitocondrial Trifuncional/deficiência
Oxirredução
Estresse Oxidativo
Placenta
Pré-Eclâmpsia/genética
Gravidez
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Fatty Acids); 2577-94-8 (arginine methyl ester); 94ZLA3W45F (Arginine); EC 1.1.1.- (3-Hydroxyacyl CoA Dehydrogenases); EC 1.1.1.211 (Long-Chain-3-Hydroxyacyl-CoA Dehydrogenase); EC 1.1.1.35 (3-Hydroxyacyl-CoA Dehydrogenase); EC 2.3.1.16 (Mitochondrial Trifunctional Protein)
[Em] Mês de entrada:1603
[Cu] Atualização por classe:161126
[Lr] Data última revisão:
161126
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:151218
[St] Status:MEDLINE


  4 / 97 MEDLINE  
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Wajner, Moacir
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[PMID]:26408230
[Au] Autor:Cecatto C; Hickmann FH; Rodrigues MD; Amaral AU; Wajner M
[Ad] Endereço:Departamento de Bioquímica, Instituto de Ciências Básicas da Saúde, Universidade Federal do Rio Grande do Sul, Porto Alegre, Brazil.
[Ti] Título:Deregulation of mitochondrial functions provoked by long-chain fatty acid accumulating in long-chain 3-hydroxyacyl-CoA dehydrogenase and mitochondrial permeability transition deficiencies in rat heart--mitochondrial permeability transition pore opening as a potential contributing pathomechanism of cardiac alterations in these disorders.
[So] Source:FEBS J;282(24):4714-26, 2015 Dec.
[Is] ISSN:1742-4658
[Cp] País de publicação:England
[La] Idioma:eng
[Ab] Resumo:Mitochondrial trifunctional protein and long-chain 3-hydroxyacyl-CoA dehydrogenase deficiencies are fatty acid oxidation disorders biochemically characterized by tissue accumulation of long-chain fatty acids and derivatives, including the monocarboxylic long-chain 3-hydroxy fatty acids (LCHFAs) 3-hydroxytetradecanoic acid (3HTA) and 3-hydroxypalmitic acid (3HPA). Patients commonly present severe cardiomyopathy for which the pathogenesis is still poorly established. We investigated the effects of 3HTA and 3HPA, the major metabolites accumulating in these disorders, on important parameters of mitochondrial homeostasis in Ca(2+) -loaded heart mitochondria. 3HTA and 3HPA significantly decreased mitochondrial membrane potential, the matrix NAD(P)H pool and Ca(2+) retention capacity, and also induced mitochondrial swelling. These fatty acids also provoked a marked decrease of ATP production reflecting severe energy dysfunction. Furthermore, 3HTA-induced mitochondrial alterations were completely prevented by the classical mitochondrial permeability transition (mPT) inhibitors cyclosporin A and ADP, as well as by ruthenium red, a Ca(2+) uptake blocker, indicating that LCHFAs induced Ca(2+)-dependent mPT pore opening. Milder effects only achieved at higher doses of LCHFAs were observed in brain mitochondria, implying a higher vulnerability of heart to these fatty acids. By contrast, 3HTA and docosanoic acids did not change mitochondrial homeostasis, indicating selective effects for monocarboxylic LCHFAs. The present data indicate that the major LCHFAs accumulating in mitochondrial trifunctional protein and long-chain 3-hydroxyacyl-CoA dehydrogenase deficiencies induce mPT pore opening, compromising Ca(2+) homeostasis and oxidative phosphorylation more intensely in the heart. It is proposed that these pathomechanisms may contribute at least in part to the severe cardiac alterations characteristic of patients affected by these diseases.
[Mh] Termos MeSH primário: Sinalização do Cálcio
3-Hidroxiacil-CoA Desidrogenase de Cadeia Longa/metabolismo
Mitocôndrias Cardíacas/metabolismo
Proteínas de Transporte da Membrana Mitocondrial/metabolismo
Ácidos Mirísticos/metabolismo
Fosforilação Oxidativa
Ácidos Palmíticos/metabolismo
[Mh] Termos MeSH secundário: Trifosfato de Adenosina/metabolismo
Animais
Bloqueadores dos Canais de Cálcio/farmacologia
Sinalização do Cálcio/efeitos dos fármacos
Cardiomiopatias/enzimologia
Cardiomiopatias/metabolismo
Permeabilidade da Membrana Celular/efeitos dos fármacos
Inibidores Enzimáticos/farmacologia
Seres Humanos
Erros Inatos do Metabolismo Lipídico/enzimologia
Erros Inatos do Metabolismo Lipídico/metabolismo
3-Hidroxiacil-CoA Desidrogenase de Cadeia Longa/deficiência
Potencial da Membrana Mitocondrial/efeitos dos fármacos
Mitocôndrias Cardíacas/efeitos dos fármacos
Mitocôndrias Cardíacas/enzimologia
Membranas Mitocondriais/efeitos dos fármacos
Membranas Mitocondriais/metabolismo
Miopatias Mitocondriais/enzimologia
Miopatias Mitocondriais/metabolismo
Dilatação Mitocondrial/efeitos dos fármacos
Proteína Mitocondrial Trifuncional/deficiência
Proteína Mitocondrial Trifuncional/metabolismo
NADP/metabolismo
Doenças do Sistema Nervoso/enzimologia
Doenças do Sistema Nervoso/metabolismo
Especificidade de Órgãos
Fosforilação Oxidativa/efeitos dos fármacos
Ratos Wistar
Rabdomiólise/enzimologia
Rabdomiólise/metabolismo
[Pt] Tipo de publicação:JOURNAL ARTICLE; RESEARCH SUPPORT, NON-U.S. GOV'T
[Nm] Nome de substância:
0 (Calcium Channel Blockers); 0 (Enzyme Inhibitors); 0 (Mitochondrial Membrane Transport Proteins); 0 (Myristic Acids); 0 (Palmitic Acids); 0 (mitochondrial permeability transition pore); 1961-72-4 (beta-hydroxymyristic acid); 2398-34-7 (3-hydroxypalmitic acid); 53-59-8 (NADP); 8L70Q75FXE (Adenosine Triphosphate); EC 1.1.1.211 (Long-Chain-3-Hydroxyacyl-CoA Dehydrogenase); EC 2.3.1.16 (Mitochondrial Trifunctional Protein)
[Em] Mês de entrada:1605
[Cu] Atualização por classe:160115
[Lr] Data última revisão:
160115
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:150927
[St] Status:MEDLINE
[do] DOI:10.1111/febs.13526


  5 / 97 MEDLINE  
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Texto completo
[PMID]:26051273
[Au] Autor:Uppala R; McKinney RW; Brant KA; Fabisiak JP; Goetzman ES
[Ad] Endereço:Department of Pediatrics, University of Pittsburgh School of Medicine, Pittsburgh, PA 15224, USA.
[Ti] Título:Nickel inhibits mitochondrial fatty acid oxidation.
[So] Source:Biochem Biophys Res Commun;463(4):806-10, 2015 Aug 07.
[Is] ISSN:1090-2104
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:Nickel exposure is associated with changes in cellular energy metabolism which may contribute to its carcinogenic properties. Here, we demonstrate that nickel strongly represses mitochondrial fatty acid oxidation-the pathway by which fatty acids are catabolized for energy-in both primary human lung fibroblasts and mouse embryonic fibroblasts. At the concentrations used, nickel suppresses fatty acid oxidation without globally suppressing mitochondrial function as evidenced by increased glucose oxidation to CO2. Pre-treatment with l-carnitine, previously shown to prevent nickel-induced mitochondrial dysfunction in neuroblastoma cells, did not prevent the inhibition of fatty acid oxidation. The effect of nickel on fatty acid oxidation occurred only with prolonged exposure (>5 h), suggesting that direct inhibition of the active sites of metabolic enzymes is not the mechanism of action. Nickel is a known hypoxia-mimetic that activates hypoxia inducible factor-1α (HIF1α). Nickel-induced inhibition of fatty acid oxidation was blunted in HIF1α knockout fibroblasts, implicating HIF1α as one contributor to the mechanism. Additionally, nickel down-regulated the protein levels of the key fatty acid oxidation enzyme very long-chain acyl-CoA dehydrogenase (VLCAD) in a dose-dependent fashion. In conclusion, inhibition of fatty acid oxidation by nickel, concurrent with increased glucose metabolism, represents a form of metabolic reprogramming that may contribute to nickel-induced carcinogenesis.
[Mh] Termos MeSH primário: Ácidos Graxos/metabolismo
Mitocôndrias/efeitos dos fármacos
Níquel/farmacologia
[Mh] Termos MeSH secundário: Western Blotting
Células Cultivadas
Seres Humanos
3-Hidroxiacil-CoA Desidrogenase de Cadeia Longa/metabolismo
Mitocôndrias/metabolismo
Oxirredução
Especificidade por Substrato
[Pt] Tipo de publicação:JOURNAL ARTICLE; RESEARCH SUPPORT, N.I.H., EXTRAMURAL
[Nm] Nome de substância:
0 (Fatty Acids); 7OV03QG267 (Nickel); EC 1.1.1.211 (Long-Chain-3-Hydroxyacyl-CoA Dehydrogenase)
[Em] Mês de entrada:1511
[Cu] Atualização por classe:161019
[Lr] Data última revisão:
161019
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:150609
[St] Status:MEDLINE


  6 / 97 MEDLINE  
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[PMID]:26024122
[Au] Autor:Polinati PP; Ilmarinen T; Trokovic R; Hyotylainen T; Otonkoski T; Suomalainen A; Skottman H; Tyni T
[Ad] Endereço:Research Program of Molecular Neurology, Biomedicum 1, University of Helsinki, Helsinki, Finland.
[Ti] Título:Patient-Specific Induced Pluripotent Stem Cell-Derived RPE Cells: Understanding the Pathogenesis of Retinopathy in Long-Chain 3-Hydroxyacyl-CoA Dehydrogenase Deficiency.
[So] Source:Invest Ophthalmol Vis Sci;56(5):3371-82, 2015 May.
[Is] ISSN:1552-5783
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:PURPOSE: Retinopathy is an important manifestation of trifunctional protein (TFP) deficiencies but not of other defects of fatty acid oxidation. The common homozygous mutation in the TFP α-subunit gene HADHA (hydroxyacyl-CoA dehydrogenase), c.1528G>C, affects the long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) activity of TFP and blindness in infancy. The pathogenesis of the retinopathy is unknown. This study aimed to utilize human induced pluripotent stem cell (hiPSC) technology to create a disease model for the disorder, and to derive clues for retinopathy pathogenesis. METHODS: We implemented hiPSC technology to generate LCHAD deficiency (LCHADD) patient-specific retinal pigment epithelial (RPE) monolayers. These patient and control RPEs were extensively characterized for function and structure, as well as for lipid composition by mass spectrometry. RESULTS: The hiPSC-derived RPE monolayers of patients and controls were functional, as they both were able to phagocytose the photoreceptor outer segments in vitro. Interestingly, the patient RPEs had intense cytoplasmic neutral lipid accumulation, and lipidomic analysis revealed an increased triglyceride accumulation. Further, patient RPEs were small and irregular in shape, and their tight junctions were disorganized. Their ultrastructure showed decreased pigmentation, few melanosomes, and more melanolysosomes. CONCLUSIONS: We demonstrate that the RPE cell model reveals novel early pathogenic changes in LCHADD retinopathy, with robust lipid accumulation, inefficient pigmentation that is evident soon after differentiation, and a defect in forming tight junctions inducing apoptosis. We propose that LCHADD-RPEs are an important model for mitochondrial TFP retinopathy, and that their early pathogenic changes contribute to infantile blindness of LCHADD.
[Mh] Termos MeSH primário: Células-Tronco Pluripotentes Induzidas/citologia
3-Hidroxiacil-CoA Desidrogenase de Cadeia Longa/deficiência
Doenças Retinianas/patologia
Epitélio Pigmentado da Retina/patologia
[Mh] Termos MeSH secundário: Biomarcadores/metabolismo
Linhagem Celular
Células Cultivadas
Células Epiteliais/metabolismo
Células Epiteliais/ultraestrutura
Seres Humanos
Imuno-Histoquímica
Lipídeos/análise
Espectrometria de Massas
Mitocôndrias/enzimologia
Doenças Retinianas/metabolismo
Epitélio Pigmentado da Retina/metabolismo
[Pt] Tipo de publicação:JOURNAL ARTICLE; RESEARCH SUPPORT, NON-U.S. GOV'T
[Nm] Nome de substância:
0 (Biomarkers); 0 (Lipids); EC 1.1.1.211 (Long-Chain-3-Hydroxyacyl-CoA Dehydrogenase)
[Em] Mês de entrada:1509
[Cu] Atualização por classe:151119
[Lr] Data última revisão:
151119
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:150530
[St] Status:MEDLINE
[do] DOI:10.1167/iovs.14-14007


  7 / 97 MEDLINE  
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[PMID]:25633398
[Au] Autor:Holub K; Camune B
[Ad] Endereço:Louise Herrington School of Nursing, Baylor University, 3700 Worth St, Dallas, TX 75246, USA. Karen_Holub@baylor.edu
[Ti] Título:Caring for the woman with acute fatty liver of pregnancy.
[So] Source:J Perinat Neonatal Nurs;29(1):32-40, 2015 Jan-Mar.
[Is] ISSN:1550-5073
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:Acute fatty liver of pregnancy, although rare, is usually a third trimester of pregnancy occurrence that may be life threatening for both the pregnant woman and the fetus. Often, the onset resembles gastroenteritis or cholecystitis and correct diagnosis is delayed. Because it can also present with preeclampsia and eclampsia, it may be mistakenly diagnosed as hemolysis, elevated liver enzymes, low platelet syndrome. This article presents diagnostic differences between liver conditions that can complicate pregnancy and management strategies for treating and maintaining the well-being of pregnant women, fetuses, and infants who are affected by acute fatty liver of pregnancy. Early recognition and rapid intervention from antepartum diagnosis through delivery and the postpartum period are required by the nursing team and medical providers to reduce maternal and neonatal morbidity and mortality.
[Mh] Termos MeSH primário: Fígado Gorduroso
Síndrome HELLP/diagnóstico
Complicações na Gravidez
[Mh] Termos MeSH secundário: Diagnóstico Tardio/efeitos adversos
Diagnóstico Tardio/prevenção & controle
Diagnóstico Diferencial
Gerenciamento Clínico
Diagnóstico Precoce
Intervenção Médica Precoce/métodos
Fígado Gorduroso/diagnóstico
Fígado Gorduroso/etiologia
Fígado Gorduroso/fisiopatologia
Feminino
Seres Humanos
Recém-Nascido
Testes de Função Hepática/métodos
3-Hidroxiacil-CoA Desidrogenase de Cadeia Longa/genética
Gravidez
Complicações na Gravidez/diagnóstico
Complicações na Gravidez/etiologia
Complicações na Gravidez/fisiopatologia
Resultado da Gravidez
[Pt] Tipo de publicação:JOURNAL ARTICLE; REVIEW
[Nm] Nome de substância:
EC 1.1.1.211 (Long-Chain-3-Hydroxyacyl-CoA Dehydrogenase)
[Em] Mês de entrada:1611
[Cu] Atualização por classe:161230
[Lr] Data última revisão:
161230
[Sb] Subgrupo de revista:N
[Da] Data de entrada para processamento:150131
[St] Status:MEDLINE
[do] DOI:10.1097/JPN.0000000000000076


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[PMID]:25269899
[Au] Autor:Yu H; Yang Z; Ding X; Wang Y; Han Y
[Ad] Endereço:Department of Obstetrics and Gynecology, Peking University Third Hospital, Beijing 100191, China.
[Ti] Título:Correlation between the different chain lengths of free fatty acid oxidation and ability of trophoblastic invasion.
[So] Source:Chin Med J (Engl);127(19):3378-82, 2014.
[Is] ISSN:0366-6999
[Cp] País de publicação:China
[La] Idioma:eng
[Ab] Resumo:BACKGROUND: Preeclampsia (PE) is associated with abnormal fatty acid beta-oxidation (FAO), especially metabolic disorders of long-chain fatty acid oxidation. The role of FAO dysfunction in inadequate invasion is unclear. The aim of this study was to explore the influence of various lengths fatty acids oxidation on invasiveness of trophoblasts. METHODS: Primary human trophoblast cells and HTR8/SVneo cells were treated with fatty acids of various lengths. Morphological changes, lipid deposition and ultrastructure changes of trophoblast cells were detected. Cells invasiveness was determined by transwell insert. CPT1, CPT2 and long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) protein expression were analyzed. The correlation between intracellular lipid droplets deposition and cells invasiveness was evaluated. RESULTS: Cells treated with long-chain fatty acids showed significant increased lipid droplets deposition, severe mitochondrial damage, decreased CPT2 and LCHAD protein expression (P < 0.05) but no significant difference in CPT1 protein expression (P > 0.05). Invasiveness of the trophoblast cells of the LC-FFA group significantly decreased (P < 0.05). Intracellular lipid droplets deposition was negatively correlated with invasivenss (R = -0.745, P < 0.05). CONCLUSION: Trophoblast cells after stimulation with long chain fatty acids exist fatty acid oxidation disorders, and reduce the ability of trophoblastic invasion.
[Mh] Termos MeSH primário: Ácidos Graxos não Esterificados/farmacologia
Trofoblastos/efeitos dos fármacos
[Mh] Termos MeSH secundário: Linhagem Celular
Células Cultivadas
Seres Humanos
Metabolismo dos Lipídeos
3-Hidroxiacil-CoA Desidrogenase de Cadeia Longa/metabolismo
[Pt] Tipo de publicação:JOURNAL ARTICLE; RESEARCH SUPPORT, NON-U.S. GOV'T
[Nm] Nome de substância:
0 (Fatty Acids, Nonesterified); EC 1.1.1.211 (Long-Chain-3-Hydroxyacyl-CoA Dehydrogenase)
[Em] Mês de entrada:1504
[Cu] Atualização por classe:141001
[Lr] Data última revisão:
141001
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:141002
[St] Status:MEDLINE


  9 / 97 MEDLINE  
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[PMID]:24780638
[Au] Autor:Martin JM; Gillingham MB; Harding CO
[Ad] Endereço:Department of Molecular & Medical Genetics, Oregon Health & Science University, 3181 SW Sam Jackson Park Road, Portland, OR 97239, USA.
[Ti] Título:Use of propofol for short duration procedures in children with long chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) or trifunctional protein (TFP) deficiencies.
[So] Source:Mol Genet Metab;112(2):139-42, 2014 Jun.
[Is] ISSN:1096-7206
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:The medication propofol, commonly used for anesthesia, has been avoided in patients with mitochondrial fatty acid oxidation disorders (FAODs) due to concerns that it contains long-chain fatty acids (LCFAs), and because of reports of severe side effects in some critically ill patients receiving high-dose propofol infusions that mimic some of the symptoms regularly found in FAOD patients. In this secondary analysis, we examined the outcomes of 8 children with long-chain 3-hydroxyacyl CoA dehydrogenase (LCHAD) deficiency or trifunctional protein (TFP) deficiency who were repeatedly sedated for an electroretinogram (ERG) as part of a longitudinal study of the progression of chorioretinopathy commonly found in this population. A total of 39 sedated ERG procedures were completed using propofol for sedation. The propofol dosing, estimated total energy needs of the subject, and inpatient dietary intake recording were completed in 32 of these procedures. The LCFAs in the propofol provided approximately 1.0% of the average total daily energy needs. The sedation with propofol resulted in no adverse side effects and was safely used in this short duration procedure.
[Mh] Termos MeSH primário: Hipnóticos e Sedativos/administração & dosagem
3-Hidroxiacil-CoA Desidrogenase de Cadeia Longa/deficiência
Proteína Mitocondrial Trifuncional/deficiência
Propofol/administração & dosagem
Doenças Retinianas/fisiopatologia
[Mh] Termos MeSH secundário: Criança
Esquema de Medicação
Eletrorretinografia
Metabolismo Energético/efeitos dos fármacos
Seres Humanos
Hipnóticos e Sedativos/efeitos adversos
3-Hidroxiacil-CoA Desidrogenase de Cadeia Longa/metabolismo
Estudos Longitudinais
Proteína Mitocondrial Trifuncional/metabolismo
Propofol/efeitos adversos
Doenças Retinianas/dietoterapia
Resultado do Tratamento
[Pt] Tipo de publicação:JOURNAL ARTICLE; RESEARCH SUPPORT, N.I.H., EXTRAMURAL; RESEARCH SUPPORT, NON-U.S. GOV'T
[Nm] Nome de substância:
0 (Hypnotics and Sedatives); EC 1.1.1.211 (Long-Chain-3-Hydroxyacyl-CoA Dehydrogenase); EC 2.3.1.16 (Mitochondrial Trifunctional Protein); YI7VU623SF (Propofol)
[Em] Mês de entrada:1501
[Cu] Atualização por classe:161019
[Lr] Data última revisão:
161019
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:140501
[St] Status:MEDLINE


  10 / 97 MEDLINE  
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[PMID]:24713206
[Au] Autor:Ding X; Yang Z; Han Y; Yu H
[Ad] Endereço:Department of Obstetrics and Gynecology, Peking University Third Hospital, Beijing 100191, PR China.
[Ti] Título:Long-chain fatty acid oxidation changes in a ß2 glycoprotein I-induced preeclampsia-like mouse model.
[So] Source:Placenta;35(6):392-7, 2014 Jun.
[Is] ISSN:1532-3102
[Cp] País de publicação:Netherlands
[La] Idioma:eng
[Ab] Resumo:INTRODUCTION: Abnormal fatty acid oxidation (FAO) and lipid metabolism have been found related to preeclampsia (PE). Antiphospholipid syndrome (APS) as a clinical risk factor for PE has also been reported with abnormal lipid metabolism. However, the role of FAO in PE accompanied with APS is unknown. We aimed to investigate long-chain FAO changes in a PE-like rodent model induced by beta 2-glycoprotein I (ß2GPI). METHODS: The PE-like model was established by injection of ß2GPI (ß2GPI group) or normal saline (control group) into C57BL/6J mice which were sacrificed on day 14 or 18 of gestation. Serum levels of anti-cardiolipin antibodies (aCL), anti-ß2GPI antibodies (aß2GPI) and serum lipids were assayed. Lipid deposition in the placenta and maternal liver was detected by lipid staining. Long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) mRNA and protein expression in the placenta and maternal liver was analyzed. RESULTS: The ß2GPI group showed PE-like symptoms including hypertension, proteinuria and adverse pregnancy outcomes. Serum aCL, aß2GPI, free fatty acid (FFA) and triglyceride (TG) levels in the ß2GPI group were significantly elevated compared with the corresponding control group (P < 0.05), while cholesterol showed no significant changes. Placenta and maternal liver fatty infiltration was found in the ß2GPI group. LCHAD mRNA and protein expression in the placenta and maternal liver in the ß2GPI group were significantly elevated compared with the corresponding control group (P < 0.05). CONCLUSION: ß2GPI can induce PE-like symptoms, elevated serum FFA and TG, and abnormal LCHAD expression in pregnant mice. Changes in long-chain FAO could be a factor linking PE and APS.
[Mh] Termos MeSH primário: Ácidos Graxos/metabolismo
Pré-Eclâmpsia/metabolismo
beta 2-Glicoproteína I/administração & dosagem
[Mh] Termos MeSH secundário: Animais
Anticorpos Antifosfolipídeos/sangue
Síndrome Antifosfolipídica/imunologia
Síndrome Antifosfolipídica/metabolismo
Cardiolipinas/imunologia
Modelos Animais de Doenças
Ácidos Graxos não Esterificados/sangue
Feminino
Hipertensão
Metabolismo dos Lipídeos
Fígado/química
Fígado/patologia
3-Hidroxiacil-CoA Desidrogenase de Cadeia Longa/análise
3-Hidroxiacil-CoA Desidrogenase de Cadeia Longa/genética
Masculino
Camundongos
Camundongos Endogâmicos C57BL
Oxirredução
Estresse Oxidativo
Placenta/química
Placenta/patologia
Pré-Eclâmpsia/imunologia
Pré-Eclâmpsia/patologia
Gravidez
Resultado da Gravidez
Proteinúria
RNA Mensageiro/análise
Triglicerídeos/sangue
beta 2-Glicoproteína I/imunologia
[Pt] Tipo de publicação:JOURNAL ARTICLE; RESEARCH SUPPORT, NON-U.S. GOV'T
[Nm] Nome de substância:
0 (Antibodies, Antiphospholipid); 0 (Cardiolipins); 0 (Fatty Acids); 0 (Fatty Acids, Nonesterified); 0 (RNA, Messenger); 0 (Triglycerides); 0 (beta 2-Glycoprotein I); EC 1.1.1.211 (Long-Chain-3-Hydroxyacyl-CoA Dehydrogenase)
[Em] Mês de entrada:1501
[Cu] Atualização por classe:140513
[Lr] Data última revisão:
140513
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:140410
[St] Status:MEDLINE



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