Base de dados : MEDLINE
Pesquisa : D02.033.415.230 [Categoria DeCS]
Referências encontradas : 737 [refinar]
Mostrando: 1 .. 10   no formato [Detalhado]

página 1 de 74 ir para página                         

  1 / 737 MEDLINE  
              next record last record
seleciona
para imprimir
Fotocópia
Texto completo
[PMID]:29031613
[Au] Autor:Majd H; King MS; Smith AC; Kunji ERS
[Ad] Endereço:Medical Research Council Mitochondrial Biology Unit, University of Cambridge, Wellcome Trust/MRC Building, Cambridge Biomedical Campus, Hills Road, Cambridge CB2 0XY, UK.
[Ti] Título:Pathogenic mutations of the human mitochondrial citrate carrier SLC25A1 lead to impaired citrate export required for lipid, dolichol, ubiquinone and sterol synthesis.
[So] Source:Biochim Biophys Acta;1859(1):1-7, 2018 01.
[Is] ISSN:0006-3002
[Cp] País de publicação:Netherlands
[La] Idioma:eng
[Ab] Resumo:Missense mutations of the human mitochondrial citrate carrier, encoded by the SLC25A1 gene, lead to an autosomal recessive neurometabolic disorder characterised by neonatal-onset encephalopathy with severe muscular weakness, intractable seizures, respiratory distress, and lack of psychomotor development, often resulting in early death. Here, we have measured the effect of all twelve known pathogenic mutations on the transport activity. The results show that nine mutations abolish transport of citrate completely, whereas the other three reduce the transport rate by >70%, indicating that impaired citrate transport is the most likely primary cause of the disease. Some mutations may be detrimental to the structure of the carrier, whereas others may impair key functional elements, such as the substrate binding site and the salt bridge network on the matrix side of the carrier. To understand the consequences of impaired citrate transport on metabolism, the substrate specificity was also determined, showing that the human citrate carrier predominantly transports citrate, isocitrate, cis-aconitate, phosphoenolpyruvate and malate. Although D-2- and L-2 hydroxyglutaric aciduria is a metabolic hallmark of the disease, it is unlikely that the citrate carrier plays a significant role in the removal of hydroxyglutarate from the cytosol for oxidation to oxoglutarate in the mitochondrial matrix. In contrast, computer simulations of central metabolism predict that the export of citrate from the mitochondrion cannot be fully compensated by other pathways, restricting the cytosolic production of acetyl-CoA that is required for the synthesis of lipids, sterols, dolichols and ubiquinone, which in turn explains the severe disease phenotypes.
[Mh] Termos MeSH primário: Proteínas de Transporte de Ânions
Ácido Cítrico/metabolismo
Simulação por Computador
Dolicol
Proteínas Mitocondriais
Modelos Biológicos
Mutação de Sentido Incorreto
Esteróis
Ubiquinona
[Mh] Termos MeSH secundário: Proteínas de Transporte de Ânions/química
Proteínas de Transporte de Ânions/genética
Proteínas de Transporte de Ânions/metabolismo
Transporte Biológico Ativo/genética
Encefalopatias Metabólicas Congênitas/enzimologia
Encefalopatias Metabólicas Congênitas/genética
Domínio Catalítico
Dolicol/biossíntese
Dolicol/química
Dolicol/genética
Seres Humanos
Proteínas Mitocondriais/química
Proteínas Mitocondriais/genética
Proteínas Mitocondriais/metabolismo
Esteróis/biossíntese
Esteróis/química
Esteróis/metabolismo
Ubiquinona/biossíntese
Ubiquinona/química
Ubiquinona/genética
[Pt] Tipo de publicação:JOURNAL ARTICLE; RESEARCH SUPPORT, NON-U.S. GOV'T
[Nm] Nome de substância:
0 (Anion Transport Proteins); 0 (Mitochondrial Proteins); 0 (Slc25a1 protein, human); 0 (Sterols); 1339-63-5 (Ubiquinone); 2067-66-5 (Dolichol); 2968PHW8QP (Citric Acid)
[Em] Mês de entrada:1803
[Cu] Atualização por classe:180309
[Lr] Data última revisão:
180309
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:171017
[St] Status:MEDLINE


  2 / 737 MEDLINE  
              first record previous record next record last record
seleciona
para imprimir
Fotocópia
Texto completo
[PMID]:27769035
[Au] Autor:Bosco M; Massarweh A; Iatmanen-Harbi S; Bouhss A; Chantret I; Busca P; Moore SE; Gravier-Pelletier C
[Ad] Endereço:Université Paris Descartes, CICB-Paris, CNRS UMR 8601, Laboratoire de Chimie et Biochimie Pharmacologiques et Toxicologiques, 45 rue des Saints-Pères, 75006, Paris, France.
[Ti] Título:Synthesis and biological evaluation of chemical tools for the study of Dolichol Linked Oligosaccharide Diphosphatase (DLODP).
[So] Source:Eur J Med Chem;125:952-964, 2017 Jan 05.
[Is] ISSN:1768-3254
[Cp] País de publicação:France
[La] Idioma:eng
[Ab] Resumo:Citronellyl- and solanesyl-based dolichol linked oligosaccharide (DLO) analogs were synthesized and tested along with undecaprenyl compounds for their ability to inhibit the release of [ H]OSP from [ H]DLO by mammalian liver DLO diphosphatase activity. Solanesyl (C45) and undecaprenyl (C55) compounds were 50-500 fold more potent than their citronellyl (C10)-based counterparts, indicating that the alkyl chain length is important for activity. The relative potency of the compounds within the citronellyl series was different to that of the solanesyl series with citronellyl diphosphate being 2 and 3 fold more potent than citronellyl-PP-GlcNAc and citronellyl-PP-GlcNAc, respectively; whereas solanesyl-PP-GlcNAc and solanesyl-PP-GlcNAc were 4 and 8 fold more potent, respectively, than solanesyl diphosphate. Undecaprenyl-PP-GlcNAc and bacterial Lipid II were 8 fold more potent than undecaprenyl diphosphate at inhibiting the DLODP assay. Therefore, at least for the more hydrophobic compounds, diphosphodiesters are more potent inhibitors of the DLODP assay than diphosphomonoesters. These results suggest that DLO rather than dolichyl diphosphate might be a preferred substrate for the DLODP activity.
[Mh] Termos MeSH primário: Dolicol/química
Oligossacarídeos/química
[Mh] Termos MeSH secundário: Animais
Fosfatos de Dolicol
Seres Humanos
Fígado/enzimologia
Monoterpenos
Diester Fosfórico Hidrolases/metabolismo
Monoéster Fosfórico Hidrolases/metabolismo
Açúcares de Poli-Isoprenil Fosfato
Fosfatos de Poli-Isoprenil
Especificidade por Substrato
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Dolichol Phosphates); 0 (Monoterpenes); 0 (Oligosaccharides); 0 (Polyisoprenyl Phosphate Sugars); 0 (Polyisoprenyl Phosphates); 2067-66-5 (Dolichol); 37247-98-6 (dolichol pyrophosphate); 60037-55-0 (solanesyl pyrophosphate); EC 3.1.3.2 (Phosphoric Monoester Hydrolases); EC 3.1.4.- (Phosphoric Diester Hydrolases)
[Em] Mês de entrada:1702
[Cu] Atualização por classe:170221
[Lr] Data última revisão:
170221
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:161022
[St] Status:MEDLINE


  3 / 737 MEDLINE  
              first record previous record next record last record
seleciona
para imprimir
Fotocópia
Texto completo
[PMID]:27480685
[Au] Autor:Kwon M; Kwon EJ; Ro DK
[Ad] Endereço:University of Calgary, Calgary, AB, Canada.
[Ti] Título:cis-Prenyltransferase and Polymer Analysis from a Natural Rubber Perspective.
[So] Source:Methods Enzymol;576:121-45, 2016.
[Is] ISSN:1557-7988
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:Dolichol and natural rubber are representative cis-polyisoprenoids in primary and secondary metabolism, respectively. Their biosynthesis is catalyzed by cis-prenyltransferase (CPT) by sequential condensations of isopentenyl diphosphates (IPPs) to a priming molecule. Although prokaryotic CPTs have been well characterized, the mechanism of eukaryotic CPTs in cis-polyisoprene biosynthesis was only recently revealed. It was shown that eukaryotes have evolved a unique protein complex, comprised of CPT and CPT-binding protein (CBP), to synthesize cis-polyisoprenoids. In the context of this new discovery, we found discrepancies in literature for CPT or CBP biochemical assays and in vivo CPT complementation using rer2 (yeast CPT) yeast mutant. Our study here shows that rer2 revertants occur at a frequency that cannot be disregarded and are likely accountable for the results that cannot be explained by the CPT/CBP heteroprotein complex model. To make a stable mutant, SRT1 gene (secondary CPT expressed at a basal level in yeast) was additionally deleted in the rer2Δ mutant background. This stable rer2Δ srt1Δ strain was then used to individually or simultaneously express Arabidopsis CPT1 (AtCPT1, At2g17570) and CBP (AtLEW1, At1G11755). We found that the simultaneous expression of Arabidopsis CPT1 and AtLEW1 effectively complements the rer2Δ srt1Δ strain, whereas the individual expression of AtCPT1 alone or AtLEW1 alone failed to rescue the yeast mutant. Microsomes from the dual expresser showed an efficient incorporation of IPPs into cis-polyisoprenoid (30% in 2h). These results showed that the CPT/CBP heteroprotein complex model is valid in Arabidopsis thaliana. Experimental details of these results are described in this methodology paper.
[Mh] Termos MeSH primário: Alquil e Aril Transferases/genética
Proteínas de Arabidopsis/genética
Arabidopsis/genética
Dimetilaliltranstransferase/genética
Dolicol/metabolismo
Proteínas de Saccharomyces cerevisiae/genética
Saccharomyces cerevisiae/genética
Transferases/genética
[Mh] Termos MeSH secundário: Alquil e Aril Transferases/metabolismo
Arabidopsis/metabolismo
Proteínas de Arabidopsis/metabolismo
Vias Biossintéticas
Dimetilaliltranstransferase/metabolismo
Dolicol/genética
Técnicas de Silenciamento de Genes
Hemiterpenos/genética
Hemiterpenos/metabolismo
Mutação
Compostos Organofosforados/metabolismo
Borracha/metabolismo
Saccharomyces cerevisiae/metabolismo
Proteínas de Saccharomyces cerevisiae/metabolismo
Metabolismo Secundário
Transferases/metabolismo
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Arabidopsis Proteins); 0 (Hemiterpenes); 0 (Organophosphorus Compounds); 0 (Saccharomyces cerevisiae Proteins); 2067-66-5 (Dolichol); 358-71-4 (isopentenyl pyrophosphate); 9006-04-6 (Rubber); EC 2.- (Transferases); EC 2.5.- (Alkyl and Aryl Transferases); EC 2.5.- (Srt1 protein, S cerevisiae); EC 2.5.1.- (cis-prenyl transferase); EC 2.5.1.1 (Dimethylallyltranstransferase); EC 2.5.1.1 (RER2 protein, S cerevisiae)
[Em] Mês de entrada:1708
[Cu] Atualização por classe:170807
[Lr] Data última revisão:
170807
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:160803
[St] Status:MEDLINE


  4 / 737 MEDLINE  
              first record previous record next record last record
seleciona
para imprimir
Fotocópia
Texto completo
[PMID]:27480077
[Au] Autor:Wheeler PG; Ng BG; Sanford L; Sutton VR; Bartholomew DW; Pastore MT; Bamshad MJ; Kircher M; Buckingham KJ; Nickerson DA; Shendure J; Freeze HH
[Ad] Endereço:Nemours Children's Clinic, Orlando, Florida.
[Ti] Título:SRD5A3-CDG: Expanding the phenotype of a congenital disorder of glycosylation with emphasis on adult onset features.
[So] Source:Am J Med Genet A;170(12):3165-3171, 2016 Dec.
[Is] ISSN:1552-4833
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:Increasing numbers of congenital disorders of glycosylation (CDG) have been reported recently resulting in an expansion of the phenotypes associated with this group of disorders. SRD5A3 codes for polyprenol reductase which converts polyprenol to dolichol. This is a major pathway for dolichol biosynthesis for N-glycosylation, O-mannosylation, C-mannosylation, and GPI anchor synthesis. We present the features of five individuals (three children and two adults) with mutations in SRD5A3 focusing on the variable eye and skin involvement. We compare that to 13 affected individuals from the literature including five adults allowing us to delineate the features that may develop over time with this disorder including kyphosis, retinitis pigmentosa, and cataracts. © 2016 Wiley Periodicals, Inc.
[Mh] Termos MeSH primário: 3-Oxo-5-alfa-Esteroide 4-Desidrogenase/genética
Defeitos Congênitos da Glicosilação/genética
Olho/fisiopatologia
Proteínas de Membrana/genética
Pele/fisiopatologia
[Mh] Termos MeSH secundário: Adulto
Criança
Defeitos Congênitos da Glicosilação/fisiopatologia
Dolicol/metabolismo
Feminino
Glicosilação
Homozigoto
Seres Humanos
Masculino
Mutação
Fenótipo
Tretinoína/análogos & derivados
Tretinoína/metabolismo
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Membrane Proteins); 2067-66-5 (Dolichol); 5688UTC01R (Tretinoin); 81485-25-8 (3,7,11,15-tetramethyl-2,4,6,10,14-hexadecapentaenoic acid); EC 1.3.99.5 (3-Oxo-5-alpha-Steroid 4-Dehydrogenase); EC 1.3.99.5 (SRD5A3 protein, human)
[Em] Mês de entrada:1710
[Cu] Atualização por classe:171016
[Lr] Data última revisão:
171016
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:160803
[St] Status:MEDLINE
[do] DOI:10.1002/ajmg.a.37875


  5 / 737 MEDLINE  
              first record previous record next record last record
seleciona
para imprimir
Fotocópia
PubMed Central Texto completo
Texto completo
[PMID]:27343064
[Au] Autor:Sabry S; Vuillaumier-Barrot S; Mintet E; Fasseu M; Valayannopoulos V; Héron D; Dorison N; Mignot C; Seta N; Chantret I; Dupré T; Moore SE
[Ad] Endereço:INSERM U1149, Faculté de Médecine Xavier Bichat, 16 rue Henri Huchard, Paris, France.
[Ti] Título:A case of fatal Type I congenital disorders of glycosylation (CDG I) associated with low dehydrodolichol diphosphate synthase (DHDDS) activity.
[So] Source:Orphanet J Rare Dis;11(1):84, 2016 06 24.
[Is] ISSN:1750-1172
[Cp] País de publicação:England
[La] Idioma:eng
[Ab] Resumo:BACKGROUND: Type I congenital disorders of glycosylation (CDG-I) are mostly complex multisystemic diseases associated with hypoglycosylated serum glycoproteins. A subgroup harbour mutations in genes necessary for the biosynthesis of the dolichol-linked oligosaccharide (DLO) precursor that is essential for protein N-glycosylation. Here, our objective was to identify the molecular origins of disease in such a CDG-Ix patient presenting with axial hypotonia, peripheral hypertonia, enlarged liver, micropenis, cryptorchidism and sensorineural deafness associated with hypo glycosylated serum glycoproteins. RESULTS: Targeted sequencing of DNA revealed a splice site mutation in intron 5 and a non-sense mutation in exon 4 of the dehydrodolichol diphosphate synthase gene (DHDDS). Skin biopsy fibroblasts derived from the patient revealed ~20 % residual DHDDS mRNA, ~35 % residual DHDDS activity, reduced dolichol-phosphate, truncated DLO and N-glycans, and an increased ratio of [2-(3)H]mannose labeled glycoprotein to [2-(3)H]mannose labeled DLO. Predicted truncated DHDDS transcripts did not complement rer2-deficient yeast. SiRNA-mediated down-regulation of DHDDS in human hepatocellular carcinoma HepG2 cells largely mirrored the biochemical phenotype of cells from the patient. The patient also harboured the homozygous ALG6(F304S) variant, which does not cause CDG but has been reported to be more frequent in PMM2-CDG patients with severe/fatal disease than in those with moderate presentations. WES did not reveal other strong candidate causal genes. CONCLUSIONS: We describe a patient presenting with severe multisystem disease associated with DHDDS deficiency. As retinitis pigmentosa is the only clinical sign in previously reported cases, this report broadens the spectrum of phenotypes associated with this condition.
[Mh] Termos MeSH primário: Alquil e Aril Transferases/metabolismo
Defeitos Congênitos da Glicosilação/enzimologia
[Mh] Termos MeSH secundário: Cromatografia em Camada Delgada
Defeitos Congênitos da Glicosilação/sangue
Defeitos Congênitos da Glicosilação/metabolismo
Dolicol/análogos & derivados
Dolicol/metabolismo
Éxons/genética
Glicoproteínas/sangue
Glicoproteínas/química
Glicoproteínas/metabolismo
Glicosilação
Células Hep G2
Seres Humanos
Recém-Nascido
Masculino
Mutação
Oligossacarídeos/metabolismo
Polissacarídeos/metabolismo
RNA Interferente Pequeno/genética
Pele/metabolismo
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Glycoproteins); 0 (Oligosaccharides); 0 (Polysaccharides); 0 (RNA, Small Interfering); 148879-95-2 (dehydrodolichol); 2067-66-5 (Dolichol); EC 2.5.- (Alkyl and Aryl Transferases); EC 2.5.1.- (dehydrodolichyl diphosphate synthetase)
[Em] Mês de entrada:1711
[Cu] Atualização por classe:171107
[Lr] Data última revisão:
171107
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:160626
[St] Status:MEDLINE
[do] DOI:10.1186/s13023-016-0468-1


  6 / 737 MEDLINE  
              first record previous record next record last record
seleciona
para imprimir
Fotocópia
Texto completo
[PMID]:27281477
[Au] Autor:Massarweh A; Bosco M; Iatmanen-Harbi S; Tessier C; Amana L; Busca P; Chantret I; Gravier-Pelletier C; Moore SE
[Ad] Endereço:INSERM U1149, Paris, France Université Denis Diderot, Paris 7, Paris, France Université Pierre et Marie Curie, Paris 6, Paris, France.
[Ti] Título:Brefeldin A promotes the appearance of oligosaccharyl phosphates derived from Glc3Man9GlcNAc2-PP-dolichol within the endomembrane system of HepG2 cells.
[So] Source:J Lipid Res;57(8):1477-91, 2016 Aug.
[Is] ISSN:1539-7262
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:We reported an oligosaccharide diphosphodolichol (DLO) diphosphatase (DLODP) that generates dolichyl-phosphate and oligosaccharyl phosphates (OSPs) from DLO in vitro. This enzyme could underlie cytoplasmic OSP generation and promote dolichyl-phosphate recycling from truncated endoplasmic reticulum (ER)-generated DLO intermediates. However, during subcellular fractionation, DLODP distribution is closer to that of a Golgi apparatus (GA) marker than those of ER markers. Here, we examined the effect of brefeldin A (BFA), which fuses the GA with the ER on OSP metabolism. In order to increase the steady state level of truncated DLO while allowing formation of mature DLO (Glc3Man9GlcNAc2-PP-dolichol), dolichyl-P-mannose Man7GlcNAc2-PP-dolichol mannosyltransferase was partially downregulated in HepG2 cells. We show that BFA provokes GA endomannosidase trimming of Glc3Man9GlcNAc2-PP-dolichol to yield a Man8GlcNAc2-PP-dolichol structure that does not give rise to cytoplasmic Man8GlcNAc2-P. BFA also strikingly increased OSP derived from mature DLO within the endomembrane system without affecting levels of Man7GlcNAc2-PP-dolichol or cytoplasmic Man7GlcNAc2-P. The BFA-provoked increase in endomembrane-situated OSP is sensitive to nocodazole, and BFA causes partial redistribution of DLODP activity from GA- to ER-containing regions of density gradients. These findings are consistent with BFA-provoked microtubule-dependent GA-to-ER transport of a previously reported DLODP that acts to generate a novel endomembrane-situated OSP population.
[Mh] Termos MeSH primário: Brefeldina A/farmacologia
Dolicol/análogos & derivados
Retículo Endoplasmático/metabolismo
Complexo de Golgi/metabolismo
Membranas Intracelulares/metabolismo
Oligossacarídeos/metabolismo
[Mh] Termos MeSH secundário: Animais
Células CHO
Cricetulus
Dolicol/metabolismo
Fosfatos de Dolicol/metabolismo
Retículo Endoplasmático/efeitos dos fármacos
Complexo de Golgi/efeitos dos fármacos
Células Hep G2
Seres Humanos
Fosfatos/metabolismo
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Dolichol Phosphates); 0 (Glc3Man9GlcNAc2-PP-Dol); 0 (Oligosaccharides); 0 (Phosphates); 12698-55-4 (dolichol monophosphate); 20350-15-6 (Brefeldin A); 2067-66-5 (Dolichol)
[Em] Mês de entrada:1709
[Cu] Atualização por classe:170906
[Lr] Data última revisão:
170906
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:160610
[St] Status:MEDLINE
[do] DOI:10.1194/jlr.M068551


  7 / 737 MEDLINE  
              first record previous record next record last record
seleciona
para imprimir
Fotocópia
Texto completo
[PMID]:27037250
[Au] Autor:Massarweh A; Bosco M; Iatmanen-Harbi S; Tessier C; Auberger N; Busca P; Chantret I; Gravier-Pelletier C; Moore SE
[Ad] Endereço:INSERM U1149, Paris, France Université Denis Diderot, Paris 7, Paris, France Université Pierre et Marie Curie, Paris 6, Paris, France.
[Ti] Título:Demonstration of an oligosaccharide-diphosphodolichol diphosphatase activity whose subcellular localization is different than those of dolichyl-phosphate-dependent enzymes of the dolichol cycle.
[So] Source:J Lipid Res;57(6):1029-42, 2016 Jun.
[Is] ISSN:1539-7262
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:Oligosaccharyl phosphates (OSPs) are hydrolyzed from oligosaccharide-diphosphodolichol (DLO) during protein N-glycosylation by an uncharacterized process. An OSP-generating activity has been reported in vitro, and here we asked if its biochemical characteristics are compatible with a role in endoplasmic reticulum (ER)-situated DLO regulation. We demonstrate a Co(2+)-dependent DLO diphosphatase (DLODP) activity that splits DLO into dolichyl phosphate and OSP. DLODP has a pH optimum of 5.5 and is inhibited by vanadate but not by NaF. Polyprenyl diphosphates inhibit [(3)H]OSP release from [(3)H]DLO, the length of their alkyl chains correlating positively with inhibition potency. The diphosphodiester GlcNAc2-PP-solanesol is hydrolyzed to yield GlcNAc2-P and inhibits [(3)H]OSP release from [(3)H]DLO more effectively than the diphosphomonoester solanesyl diphosphate. During subcellular fractionation of liver homogenates, DLODP codistributes with microsomal markers, and density gradient centrifugation revealed that the distribution of DLODP is closer to that of Golgi apparatus-situated UDP-galactose glycoprotein galactosyltransferase than those of dolichyl-P-dependent glycosyltransferases required for DLO biosynthesis in the ER. Therefore, a DLODP activity showing selectivity toward lipophilic diphosphodiesters such as DLO, and possessing properties distinct from other lipid phosphatases, is identified. Separate subcellular locations for DLODP action and DLO biosynthesis may be required to prevent uncontrolled DLO destruction.
[Mh] Termos MeSH primário: Dolicol/metabolismo
Oligossacarídeos/metabolismo
Pirofosfatases/metabolismo
[Mh] Termos MeSH secundário: Dolicol/química
Fosfatos de Dolicol/química
Fosfatos de Dolicol/metabolismo
Retículo Endoplasmático/química
Retículo Endoplasmático/metabolismo
Glicosilação
Complexo de Golgi/química
Complexo de Golgi/metabolismo
Células Hep G2
Seres Humanos
Fígado/química
Fígado/metabolismo
Oligossacarídeos/química
Fosfatos de Poli-Isoprenil/química
Fosfatos de Poli-Isoprenil/metabolismo
Pirofosfatases/química
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Dolichol Phosphates); 0 (Oligosaccharides); 0 (Polyisoprenyl Phosphates); 12698-55-4 (dolichol monophosphate); 2067-66-5 (Dolichol); 60037-55-0 (solanesyl pyrophosphate); EC 3.6.1.- (Pyrophosphatases); EC 3.6.1.44 (oligosaccharide-diphosphodolichol pyrophosphatase)
[Em] Mês de entrada:1708
[Cu] Atualização por classe:170825
[Lr] Data última revisão:
170825
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:160403
[St] Status:MEDLINE
[do] DOI:10.1194/jlr.M067330


  8 / 737 MEDLINE  
              first record previous record next record last record
seleciona
para imprimir
Fotocópia
Texto completo
[PMID]:27009761
[Au] Autor:Opperdoes FR; Butenko A; Flegontov P; Yurchenko V; Lukes J
[Ad] Endereço:de Duve Institute, Université Catholique de Louvain, Brussels, B-1200, Belgium.
[Ti] Título:Comparative Metabolism of Free-living Bodo saltans and Parasitic Trypanosomatids.
[So] Source:J Eukaryot Microbiol;63(5):657-78, 2016 Sep.
[Is] ISSN:1550-7408
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:Comparison of the genomes of free-living Bodo saltans and those of parasitic trypanosomatids reveals that the transition from a free-living to a parasitic life style has resulted in the loss of approximately 50% of protein-coding genes. Despite this dramatic reduction in genome size, B. saltans and trypanosomatids still share a significant number of common metabolic traits: glycosomes; a unique set of the pyrimidine biosynthetic pathway genes; an ATP-PFK which is homologous to the bacterial PPi -PFKs rather than to the canonical eukaryotic ATP-PFKs; an alternative oxidase; three phosphoglycerate kinases and two GAPDH isoenzymes; a pyruvate kinase regulated by fructose-2,6-bisphosphate; trypanothione as a substitute for glutathione; synthesis of fatty acids via a unique set of elongase enzymes; and a mitochondrial acetate:succinate coenzyme A transferase. B. saltans has lost the capacity to synthesize ubiquinone. Among genes that are present in B. saltans and lost in all trypanosomatids are those involved in the degradation of mureine, tryptophan and lysine. Novel acquisitions of trypanosomatids are components of pentose sugar metabolism, pteridine reductase and bromodomain-factor proteins. In addition, only the subfamily Leishmaniinae has acquired a gene for catalase and the capacity to convert diaminopimelic acid to lysine.
[Mh] Termos MeSH primário: Kinetoplastida/genética
Kinetoplastida/metabolismo
Trypanosomatina/genética
Trypanosomatina/metabolismo
[Mh] Termos MeSH secundário: Aminoácidos/metabolismo
Bactérias/genética
Bactérias/metabolismo
Metabolismo dos Carboidratos
Coenzimas/metabolismo
Dolicol/metabolismo
Ergosterol/biossíntese
Eucariotos/genética
Eucariotos/metabolismo
Ácido Fólico/metabolismo
Genes de Protozoários/genética
Gluconeogênese
Glicólise
Kinetoplastida/enzimologia
Metabolismo dos Lipídeos
Ácido Mevalônico/metabolismo
Microcorpos/metabolismo
Mitocôndrias/enzimologia
Mitocôndrias/metabolismo
Oxirredutases/metabolismo
Via de Pentose Fosfato
Peroxissomos/metabolismo
Fosfolipídeos/metabolismo
Poliaminas/metabolismo
Prenilação de Proteína
Proteínas de Protozoários/genética
Purinas/biossíntese
Purinas/metabolismo
Pirimidinas/biossíntese
Pirimidinas/metabolismo
Espécies Reativas de Oxigênio
Trypanosomatina/enzimologia
Ubiquinona/metabolismo
Ureia/metabolismo
Vitaminas/metabolismo
[Pt] Tipo de publicação:JOURNAL ARTICLE; REVIEW
[Nm] Nome de substância:
0 (Amino Acids); 0 (Coenzymes); 0 (Phospholipids); 0 (Polyamines); 0 (Protozoan Proteins); 0 (Purines); 0 (Pyrimidines); 0 (Reactive Oxygen Species); 0 (Vitamins); 1339-63-5 (Ubiquinone); 2067-66-5 (Dolichol); 8W8T17847W (Urea); 935E97BOY8 (Folic Acid); EC 1.- (Oxidoreductases); K8CXK5Q32L (pyrimidine); S5UOB36OCZ (Mevalonic Acid); W60KTZ3IZY (purine); Z30RAY509F (Ergosterol)
[Em] Mês de entrada:1708
[Cu] Atualização por classe:170828
[Lr] Data última revisão:
170828
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:160325
[St] Status:MEDLINE
[do] DOI:10.1111/jeu.12315


  9 / 737 MEDLINE  
              first record previous record next record last record
seleciona
para imprimir
Fotocópia
Texto completo
[PMID]:26968401
[Au] Autor:Cavallini G; Sgarbossa A; Parentini I; Bizzarri R; Donati A; Lenci F; Bergamini E
[Ad] Endereço:Interdepartmental Research Centre on Biology and Pathology of Aging, University of Pisa, Via Roma 55, 56126, Pisa, Italy. gabriella.cavallini@med.unipi.it.
[Ti] Título:Dolichol: A Component of the Cellular Antioxidant Machinery.
[So] Source:Lipids;51(4):477-86, 2016 Apr.
[Is] ISSN:1558-9307
[Cp] País de publicação:Germany
[La] Idioma:eng
[Ab] Resumo:Dolichol, an end product of the mevalonate pathway, has been proposed as a biomarker of aging, but its biological role, not to mention its catabolism, has not been fully understood. UV-B radiation was used to induce oxidative stress in isolated rat hepatocytes by the collagenase method. Effects on dolichol, phospholipid-bound polyunsaturated fatty acids (PL-PUFA) and known lipid soluble antioxidants [coenzyme Q (CoQ) and α-tocopherol] were studied. The increase in oxidative stress was detected by a probe sensitive to reactive oxygen species (ROS). Peroxidation of lipids was assessed by measuring the release of thiobarbituric acid reactive substances (TBARS). Dolichol, CoQ, and α-tocopherol were assessed by high-pressure liquid chromatography (HPLC), PL-PUFA by gas-liquid chromatography (GC). UV-B radiation caused an immediate increase in ROS as well as lipid peroxidation and a simultaneous decrease in the levels of dolichol and lipid soluble antioxidants. Decrease in dolichol paralleled changes in CoQ levels and was smaller to that in α-tocopherol. The addition of mevinolin, a competitive inhibitor of the enzyme 3-hydroxy-3-methylglutaryl CoA reductase (HMG-CoAR), magnified the loss of dolichol and was associated with an increase in TBARS production. Changes in PL-PUFA were minor. These findings highlight that oxidative stress has very early and similar effects on dolichol and lipid soluble antioxidants. Lower levels of dolichol are associated with enhanced peroxidation of lipids, which suggest that dolichol may have a protective role in the antioxidant machinery of cell membranes and perhaps be a key to understanding some adverse effects of statin therapy.
[Mh] Termos MeSH primário: Antioxidantes/análise
Dolicol/análise
Hepatócitos/efeitos da radiação
Raios Ultravioleta/efeitos adversos
[Mh] Termos MeSH secundário: Envelhecimento/metabolismo
Animais
Células Cultivadas
Cromatografia Líquida/métodos
Hepatócitos/citologia
Peroxidação de Lipídeos/efeitos da radiação
Masculino
Ratos
Espécies Reativas de Oxigênio/metabolismo
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Antioxidants); 0 (Reactive Oxygen Species); 2067-66-5 (Dolichol)
[Em] Mês de entrada:1612
[Cu] Atualização por classe:171105
[Lr] Data última revisão:
171105
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:160313
[St] Status:MEDLINE
[do] DOI:10.1007/s11745-016-4137-x


  10 / 737 MEDLINE  
              first record previous record
seleciona
para imprimir
Fotocópia
PubMed Central Texto completo
Texto completo
[PMID]:26912795
[Au] Autor:DeRossi C; Vacaru A; Rafiq R; Cinaroglu A; Imrie D; Nayar S; Baryshnikova A; Milev MP; Stanga D; Kadakia D; Gao N; Chu J; Freeze HH; Lehrman MA; Sacher M; Sadler KC
[Ad] Endereço:Department of Medicine, Division of Liver Diseases, Icahn School of Medicine at Mount Sinai, New York, NY 10029 Department of Developmental and Regenerative Biology, Icahn School of Medicine at Mount Sinai, New York, NY 10029.
[Ti] Título:trappc11 is required for protein glycosylation in zebrafish and humans.
[So] Source:Mol Biol Cell;27(8):1220-34, 2016 Apr 15.
[Is] ISSN:1939-4586
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:Activation of the unfolded protein response (UPR) can be either adaptive or pathological. We term the pathological UPR that causes fatty liver disease a "stressed UPR." Here we investigate the mechanism of stressed UPR activation in zebrafish bearing a mutation in thetrappc11gene, which encodes a component of the transport protein particle (TRAPP) complex.trappc11mutants are characterized by secretory pathway defects, reflecting disruption of the TRAPP complex. In addition, we uncover a defect in protein glycosylation intrappc11mutants that is associated with reduced levels of lipid-linked oligosaccharides (LLOs) and compensatory up-regulation of genes in the terpenoid biosynthetic pathway that produces the LLO anchor dolichol. Treating wild-type larvae with terpenoid or LLO synthesis inhibitors phenocopies the stressed UPR seen intrappc11mutants and is synthetically lethal withtrappc11mutation. We propose that reduced LLO level causing hypoglycosylation is a mechanism of stressed UPR induction intrappc11mutants. Of importance, in human cells, depletion of TRAPPC11, but not other TRAPP components, causes protein hypoglycosylation, and lipid droplets accumulate in fibroblasts from patients with theTRAPPC11mutation. These data point to a previously unanticipated and conserved role for TRAPPC11 in LLO biosynthesis and protein glycosylation in addition to its established function in vesicle trafficking.
[Mh] Termos MeSH primário: Oligossacarídeos/metabolismo
Resposta a Proteínas não Dobradas
Proteínas de Transporte Vesicular/metabolismo
Proteínas de Peixe-Zebra/metabolismo
[Mh] Termos MeSH secundário: Animais
Animais Geneticamente Modificados
Atorvastatina Cálcica/farmacologia
Dolicol/biossíntese
Dolicol/genética
Glicosilação
Complexo de Golgi/genética
Complexo de Golgi/metabolismo
Complexo de Golgi/secreção
Seres Humanos
Larva/efeitos dos fármacos
Larva/metabolismo
Lipídeos/química
Fígado/metabolismo
Fígado/patologia
Mutação
Oligossacarídeos/química
Terpenos/metabolismo
Terpenos/farmacologia
Resposta a Proteínas não Dobradas/efeitos dos fármacos
Resposta a Proteínas não Dobradas/genética
Proteínas de Transporte Vesicular/genética
Peixe-Zebra/genética
Proteínas de Peixe-Zebra/genética
[Pt] Tipo de publicação:JOURNAL ARTICLE; RESEARCH SUPPORT, N.I.H., EXTRAMURAL; RESEARCH SUPPORT, NON-U.S. GOV'T
[Nm] Nome de substância:
0 (Lipids); 0 (Oligosaccharides); 0 (TRAPPC11 protein, human); 0 (TRAPPC11 protein, zebrafish); 0 (Terpenes); 0 (Vesicular Transport Proteins); 0 (Zebrafish Proteins); 2067-66-5 (Dolichol); 48A5M73Z4Q (Atorvastatin Calcium)
[Em] Mês de entrada:1701
[Cu] Atualização por classe:170220
[Lr] Data última revisão:
170220
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:160226
[St] Status:MEDLINE
[do] DOI:10.1091/mbc.E15-08-0557



página 1 de 74 ir para página                         
   


Refinar a pesquisa
  Base de dados : MEDLINE Formulário avançado   

    Pesquisar no campo  
1  
2
3
 
           



Search engine: iAH v2.6 powered by WWWISIS

BIREME/OPAS/OMS - Centro Latino-Americano e do Caribe de Informação em Ciências da Saúde