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[PMID]:28470336
[Au] Autor:Otori K; Tanabe N; Maruyama T; Sato S; Yanagisawa S; Tamoi M; Shigeoka S
[Ad] Endereço:Department of Advanced Bioscience, Faculty of Agriculture, Kindai University, Nakamachi, Nara, 631-8505, Japan.
[Ti] Título:Enhanced photosynthetic capacity increases nitrogen metabolism through the coordinated regulation of carbon and nitrogen assimilation in Arabidopsis thaliana.
[So] Source:J Plant Res;130(5):909-927, 2017 Sep.
[Is] ISSN:1618-0860
[Cp] País de publicação:Japan
[La] Idioma:eng
[Ab] Resumo:Plant growth and productivity depend on interactions between the metabolism of carbon and nitrogen. The sensing ability of internal carbon and nitrogen metabolites (the C/N balance) enables plants to regulate metabolism and development. In order to investigate the effects of an enhanced photosynthetic capacity on the metabolism of carbon and nitrogen in photosynthetically active tissus (source leaves), we herein generated transgenic Arabidopsis thaliana plants (ApFS) that expressed cyanobacterial fructose-1,6-/sedoheptulose-1,7-bisphosphatase in their chloroplasts. The phenotype of ApFS plants was indistinguishable from that of wild-type plants at the immature stage. However, as plants matured, the growth of ApFS plants was superior to that of wild-type plants. Starch levels were higher in ApFS plants than in wild-type plants at 2 and 5 weeks. Sucrose levels were also higher in ApFS plants than in wild-type plants, but only at 5 weeks. On the other hand, the contents of various free amino acids were lower in ApFS plants than in wild-type plants at 2 weeks, but were similar at 5 weeks. The total C/N ratio was the same in ApFS plants and wild-type plants, whereas nitrite levels increased in parallel with elevations in nitrate reductase activity at 5 weeks in ApFS plants. These results suggest that increases in the contents of photosynthetic intermediates at the early growth stage caused a temporary imbalance in the free-C/free-N ratio and, thus, the feedback inhibition of the expression of genes involved in the Calvin cycle and induction of the expression of those involved in nitrogen metabolism due to supply deficient free amino acids for maintenance of the C/N balance in source leaves of ApFS plants.
[Mh] Termos MeSH primário: Arabidopsis/fisiologia
Carbono/metabolismo
Nitrogênio/metabolismo
Fotossíntese
[Mh] Termos MeSH secundário: Arabidopsis/genética
Arabidopsis/crescimento & desenvolvimento
Proteínas de Arabidopsis/genética
Proteínas de Arabidopsis/metabolismo
Cloroplastos/enzimologia
Frutose-Bifosfatase/genética
Frutose-Bifosfatase/metabolismo
Fenótipo
Monoéster Fosfórico Hidrolases/genética
Monoéster Fosfórico Hidrolases/metabolismo
Plantas Geneticamente Modificadas
Amido/metabolismo
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Arabidopsis Proteins); 7440-44-0 (Carbon); 9005-25-8 (Starch); EC 3.1.3.11 (Fructose-Bisphosphatase); EC 3.1.3.2 (Phosphoric Monoester Hydrolases); EC 3.1.3.37 (sedoheptulose-bisphosphatase); N762921K75 (Nitrogen)
[Em] Mês de entrada:1712
[Cu] Atualização por classe:171228
[Lr] Data última revisão:
171228
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170505
[St] Status:MEDLINE
[do] DOI:10.1007/s10265-017-0950-4


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[PMID]:28934464
[Au] Autor:Asada R; Umeda M; Adachi A; Senmatsu S; Abe T; Iwasaki H; Ohta K; Hoffman CS; Hirota K
[Ad] Endereço:Department of Chemistry, Graduate School of Science and Engineering, Tokyo Metropolitan University, Minamiosawa 1-1, Hachioji-shi, Tokyo 192-0397, Japan.
[Ti] Título:Recruitment and delivery of the fission yeast Rst2 transcription factor via a local genome structure counteracts repression by Tup1-family corepressors.
[So] Source:Nucleic Acids Res;45(16):9361-9371, 2017 Sep 19.
[Is] ISSN:1362-4962
[Cp] País de publicação:England
[La] Idioma:eng
[Ab] Resumo:Transcription factors (TFs) determine the transcription activity of target genes and play a central role in controlling the transcription in response to various environmental stresses. Three dimensional genome structures such as local loops play a fundamental role in the regulation of transcription, although the link between such structures and the regulation of TF binding to cis-regulatory elements remains to be elucidated. Here, we show that during transcriptional activation of the fission yeast fbp1 gene, binding of Rst2 (a critical C2H2 zinc-finger TF) is mediated by a local loop structure. During fbp1 activation, Rst2 is first recruited to upstream-activating sequence 1 (UAS1), then it subsequently binds to UAS2 (a critical cis-regulatory site located approximately 600 base pairs downstream of UAS1) through a loop structure that brings UAS1 and UAS2 into spatially close proximity. Tup11/12 (the Tup-family corepressors) suppress direct binding of Rst2 to UAS2, but this suppression is counteracted by the recruitment of Rst2 at UAS1 and following delivery to UAS2 through a loop structure. These data demonstrate a previously unappreciated mechanism for the recruitment and expansion of TF-DNA interactions within a promoter mediated by local three-dimensional genome structures and for timely TF-binding via counteractive regulation by the Tup-family corepressors.
[Mh] Termos MeSH primário: Frutose-Bifosfatase/genética
Regulação Fúngica da Expressão Gênica
Regiões Promotoras Genéticas
Proteínas Repressoras/metabolismo
Proteínas de Schizosaccharomyces pombe/metabolismo
Schizosaccharomyces/genética
Fatores de Transcrição/metabolismo
[Mh] Termos MeSH secundário: Frutose-Bifosfatase/biossíntese
Genoma Fúngico
Conformação de Ácido Nucleico
Motivos de Nucleotídeos
Ligação Proteica
Schizosaccharomyces/metabolismo
Ativação Transcricional
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (RST2 protein, S pombe); 0 (Repressor Proteins); 0 (Schizosaccharomyces pombe Proteins); 0 (Transcription Factors); 0 (Tup11 protein, S pombe); 0 (Tup12 protein, S pombe); EC 3.1.3.11 (Fructose-Bisphosphatase)
[Em] Mês de entrada:1710
[Cu] Atualização por classe:171024
[Lr] Data última revisão:
171024
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170922
[St] Status:MEDLINE
[do] DOI:10.1093/nar/gkx555


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[PMID]:28882541
[Au] Autor:Yuan M; Vásquez-Valdivieso MG; McNae IW; Michels PAM; Fothergill-Gilmore LA; Walkinshaw MD
[Ad] Endereço:Centre for Translational and Chemical Biology, School of Biological Sciences, University of Edinburgh, Michael Swann Building, Max Born Crescent, Edinburgh EH9 3BF, UK.
[Ti] Título:Structures of Leishmania Fructose-1,6-Bisphosphatase Reveal Species-Specific Differences in the Mechanism of Allosteric Inhibition.
[So] Source:J Mol Biol;429(20):3075-3089, 2017 Oct 13.
[Is] ISSN:1089-8638
[Cp] País de publicação:England
[La] Idioma:eng
[Ab] Resumo:The gluconeogenic enzyme fructose-1,6-bisphosphatase has been proposed as a potential drug target against Leishmania parasites that cause up to 20,000-30,000 deaths annually. A comparison of three crystal structures of Leishmania major fructose-1,6-bisphosphatase (LmFBPase) along with enzyme kinetic data show how AMP acts as an allosteric inhibitor and provides insight into its metal-dependent reaction mechanism. The crystal structure of the apoenzyme form of LmFBPase is a homotetramer in which the dimer of dimers adopts a planar conformation with disordered "dynamic loops". The structure of LmFBPase, complexed with manganese and its catalytic product phosphate, shows the dynamic loops locked into the active sites. A third crystal structure of LmFBPase complexed with its allosteric inhibitor AMP shows an inactive form of the tetramer, in which the dimer pairs are rotated by 18° relative to each other. The three structures suggest an allosteric mechanism in which AMP binding triggers a rearrangement of hydrogen bonds across the large and small interfaces. Retraction of the "effector loop" required for AMP binding releases the side chain of His23 from the dimer-dimer interface. This is coupled with a flip of the side chain of Arg48 which ties down the key catalytic dynamic loop in a disengaged conformation and also locks the tetramer in an inactive rotated T-state. The structure of the effector site of LmFBPase shows different structural features compared with human FBPases, thereby offering a potential and species-specific drug target.
[Mh] Termos MeSH primário: Monofosfato de Adenosina/metabolismo
Frutose-Bifosfatase/antagonistas & inibidores
Frutose-Bifosfatase/química
Leishmania major/enzimologia
[Mh] Termos MeSH secundário: Regulação Alostérica
Coenzimas
Cristalografia por Raios X
Inibidores Enzimáticos
Seres Humanos
Cinética
Manganês/metabolismo
Modelos Moleculares
Ligação Proteica
Conformação Proteica
Multimerização Proteica
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Coenzymes); 0 (Enzyme Inhibitors); 415SHH325A (Adenosine Monophosphate); 42Z2K6ZL8P (Manganese); EC 3.1.3.11 (Fructose-Bisphosphatase)
[Em] Mês de entrada:1710
[Cu] Atualização por classe:171022
[Lr] Data última revisão:
171022
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170909
[St] Status:MEDLINE


  4 / 2184 MEDLINE  
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[PMID]:28720574
[Au] Autor:Jin X; Pan Y; Wang L; Ma T; Zhang L; Tang AH; Billadeau DD; Wu H; Huang H
[Ad] Endereço:Department of Digestive Surgical Oncology, Union Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan, Hubei, China.
[Ti] Título:Fructose-1,6-bisphosphatase Inhibits ERK Activation and Bypasses Gemcitabine Resistance in Pancreatic Cancer by Blocking IQGAP1-MAPK Interaction.
[So] Source:Cancer Res;77(16):4328-4341, 2017 Aug 15.
[Is] ISSN:1538-7445
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:Dysregulation of the MAPK pathway correlates with progression of pancreatic ductal adenocarcinoma (PDAC) progression. IQ motif containing GTPase-activating protein 1 (IQGAP1) is a MAPK scaffold that directly regulates the activation of RAF, MEK, and ERK. Fructose-1,6-bisphosphatase (FBP1), a key enzyme in gluconeogenesis, is transcriptionally downregulated in various cancers, including PDAC. Here, we demonstrate that FBP1 acts as a negative modulator of the IQGAP1-MAPK signaling axis in PDAC cells. FBP1 binding to the WW domain of IQGAP1 impeded IQGAP1-dependent ERK1/2 phosphorylation (pERK1/2) in a manner independent of FBP1 enzymatic activity. Conversely, decreased FBP1 expression induced pERK1/2 levels in PDAC cell lines and correlated with increased pERK1/2 levels in patient specimens. Treatment with gemcitabine caused undesirable activation of ERK1/2 in PDAC cells, but cotreatment with the FBP1-derived small peptide inhibitor FBP1 E4 overcame gemcitabine-induced ERK activation, thereby increasing the anticancer efficacy of gemcitabine in PDAC. These findings identify a primary mechanism of resistance of PDAC to standard therapy and suggest that the FBP1-IQGAP1-ERK1/2 signaling axis can be targeted for effective treatment of PDAC. .
[Mh] Termos MeSH primário: Antimetabólitos Antineoplásicos/farmacologia
Carcinoma Ductal Pancreático/tratamento farmacológico
Carcinoma Ductal Pancreático/genética
Desoxicitidina/análogos & derivados
Frutose-Bifosfatase/metabolismo
Sistema de Sinalização das MAP Quinases/efeitos dos fármacos
Neoplasias Pancreáticas/tratamento farmacológico
Neoplasias Pancreáticas/genética
Proteínas Ativadoras de ras GTPase/antagonistas & inibidores
[Mh] Termos MeSH secundário: Linhagem Celular Tumoral
Desoxicitidina/farmacologia
Resistência a Medicamentos Antineoplásicos
Frutose-Bifosfatase/genética
Seres Humanos
Neoplasias Pancreáticas/metabolismo
Transdução de Sinais
Transfecção
Proteínas Ativadoras de ras GTPase/metabolismo
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Antimetabolites, Antineoplastic); 0 (IQ motif containing GTPase activating protein 1); 0 (ras GTPase-Activating Proteins); 0W860991D6 (Deoxycytidine); B76N6SBZ8R (gemcitabine); EC 3.1.3.11 (Fructose-Bisphosphatase)
[Em] Mês de entrada:1709
[Cu] Atualização por classe:170929
[Lr] Data última revisão:
170929
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170720
[St] Status:MEDLINE
[do] DOI:10.1158/0008-5472.CAN-16-3143


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[PMID]:28624442
[Au] Autor:Dai J; Ji Y; Wang W; Kim D; Fai LY; Wang L; Luo J; Zhang Z
[Ad] Endereço:Department of Toxicology and Cancer Biology, 1095 Veterans Drive, University of Kentucky, Lexington, KY 40536, USA.
[Ti] Título:Loss of fructose-1,6-bisphosphatase induces glycolysis and promotes apoptosis resistance of cancer stem-like cells: an important role in hexavalent chromium-induced carcinogenesis.
[So] Source:Toxicol Appl Pharmacol;331:164-173, 2017 Sep 15.
[Is] ISSN:1096-0333
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:Hexavalent chromium (Cr(VI)) compounds are confirmed human carcinogens for lung cancer. Our previous studies has demonstrated that chronic exposure of human bronchial epithelial BEAS-2B cells to low dose of Cr(VI) causes malignant cell transformation. The acquisition of cancer stem cell-like properties is involved in the initiation of cancers. The present study has observed that a small population of cancer stem-like cells (BEAS-2B-Cr-CSC) exists in the Cr(VI)-transformed cells (BEAS-2B-Cr). Those BEAS-2B-Cr-CSC exhibit extremely reduced capability of generating reactive oxygen species (ROS) and apoptosis resistance. BEAS-2B-Cr-CSC are metabolic inactive as evidenced by reductions in oxygen consumption, glucose uptake, ATP production, and lactate production. Most importantly, BEAS-2B-Cr-CSC are more tumorigenic with high levels of cell self-renewal genes, Notch1 and p21. Further study has found that fructose-1,6-bisphosphatase (FBP1), an rate-limiting enzyme driving glyconeogenesis, was lost in BEAS-2B-Cr-CSC. Forced expression of FBP1 in BEAS-2B-Cr-CSC restored ROS generation, resulting in increased apoptosis, leading to inhibition of tumorigenesis. In summary, the present study suggests that loss of FBP1 is a critical event in tumorigenesis of Cr(VI)-transformed cells.
[Mh] Termos MeSH primário: Apoptose/efeitos dos fármacos
Carcinogênese/efeitos dos fármacos
Cromo/toxicidade
Frutose-Bifosfatase/metabolismo
Glicólise/efeitos dos fármacos
Células-Tronco Neoplásicas/efeitos dos fármacos
[Mh] Termos MeSH secundário: Animais
Apoptose/fisiologia
Carcinogênese/metabolismo
Linhagem Celular
Linhagem Celular Transformada
Células Epiteliais/efeitos dos fármacos
Células Epiteliais/metabolismo
Deficiência de Frutose-1,6-Difosfatase/metabolismo
Glicólise/fisiologia
Seres Humanos
Camundongos
Camundongos Nus
Células-Tronco Neoplásicas/metabolismo
Mucosa Respiratória/efeitos dos fármacos
Mucosa Respiratória/metabolismo
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0R0008Q3JB (Chromium); 18540-29-9 (chromium hexavalent ion); EC 3.1.3.11 (Fructose-Bisphosphatase)
[Em] Mês de entrada:1709
[Cu] Atualização por classe:170904
[Lr] Data última revisão:
170904
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170619
[St] Status:MEDLINE


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[PMID]:28594867
[Au] Autor:Tiruveedula GSS; Wangikar PP
[Ad] Endereço:Department of Chemical Engineering, National Institute of Technology Karnataka, Surathkal, Mangalore, India.
[Ti] Título:Gene essentiality, conservation index and co-evolution of genes in cyanobacteria.
[So] Source:PLoS One;12(6):e0178565, 2017.
[Is] ISSN:1932-6203
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:Cyanobacteria, a group of photosynthetic prokaryotes, dominate the earth with ~ 1015 g wet biomass. Despite diversity in habitats and an ancient origin, cyanobacterial phylum has retained a significant core genome. Cyanobacteria are being explored for direct conversion of solar energy and carbon dioxide into biofuels. For this, efficient cyanobacterial strains will need to be designed via metabolic engineering. This will require identification of target knockouts to channelize the flow of carbon toward the product of interest while minimizing deletions of essential genes. We propose "Gene Conservation Index" (GCI) as a quick measure to predict gene essentiality in cyanobacteria. GCI is based on phylogenetic profile of a gene constructed with a reduced dataset of cyanobacterial genomes. GCI is the percentage of organism clusters in which the query gene is present in the reduced dataset. Of the 750 genes deemed to be essential in the experimental study on S. elongatus PCC 7942, we found 494 to be conserved across the phylum which largely comprise of the essential metabolic pathways. On the contrary, the conserved but non-essential genes broadly comprise of genes required under stress conditions. Exceptions to this rule include genes such as the glycogen synthesis and degradation enzymes, deoxyribose-phosphate aldolase (DERA), glucose-6-phosphate 1-dehydrogenase (zwf) and fructose-1,6-bisphosphatase class1, which are conserved but non-essential. While the essential genes are to be avoided during gene knockout studies as potentially lethal deletions, the non-essential but conserved set of genes could be interesting targets for metabolic engineering. Further, we identify clusters of co-evolving genes (CCG), which provide insights that may be useful in annotation. Principal component analysis (PCA) plots of the CCGs are demonstrated as data visualization tools that are complementary to the conventional heatmaps. Our dataset consists of phylogenetic profiles for 23,643 non-redundant cyanobacterial genes. We believe that the data and the analysis presented here will be a great resource to the scientific community interested in cyanobacteria.
[Mh] Termos MeSH primário: Cianobactérias/genética
Evolução Molecular
[Mh] Termos MeSH secundário: Aldeído Liases/genética
Aldeído Liases/metabolismo
Proteínas de Bactérias/genética
Proteínas de Bactérias/metabolismo
Cianobactérias/classificação
Cianobactérias/metabolismo
Frutose-Bifosfatase/genética
Frutose-Bifosfatase/metabolismo
Glucosefosfato Desidrogenase/genética
Glucosefosfato Desidrogenase/metabolismo
Filogenia
Análise de Componente Principal
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Bacterial Proteins); EC 1.1.1.49 (Glucosephosphate Dehydrogenase); EC 3.1.3.11 (Fructose-Bisphosphatase); EC 4.1.2.- (Aldehyde-Lyases); EC 4.1.2.4 (deoxyribose-phosphate aldolase)
[Em] Mês de entrada:1709
[Cu] Atualização por classe:170919
[Lr] Data última revisão:
170919
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170609
[St] Status:MEDLINE
[do] DOI:10.1371/journal.pone.0178565


  7 / 2184 MEDLINE  
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[PMID]:28542187
[Au] Autor:Schabort DTWP; Kilian SG; du Preez JC
[Ad] Endereço:Department of Microbial, Biochemical and Food Biotechnology, University of the Free State, Bloemfontein, South Africa.
[Ti] Título:Elucidation of new condition-dependent roles for fructose-1,6-bisphosphatase linked to cofactor balances.
[So] Source:PLoS One;12(5):e0177319, 2017.
[Is] ISSN:1932-6203
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:The cofactor balances in metabolism is of paramount importance in the design of a metabolic engineering strategy and understanding the regulation of metabolism in general. ATP, NAD+ and NADP+ balances are central players linking the various fluxes in central metabolism as well as biomass formation. NADP+ is especially important in the metabolic engineering of yeasts for xylose fermentation, since NADPH is required by most yeasts in the initial step of xylose utilisation, including the fast-growing Kluyveromyces marxianus. In this simulation study of yeast metabolism, the complex interplay between these cofactors was investigated; in particular, how they may affect the possible roles of fructose-1,6-bisphosphatase, the pentose phosphate pathway, glycerol production and the pyruvate dehydrogenase bypass. Using flux balance analysis, it was found that the potential role of fructose-1,6-bisphosphatase was highly dependent on the cofactor specificity of the oxidative pentose phosphate pathway and on the carbon source. Additionally, the excessive production of ATP under certain conditions might be involved in some of the phenomena observed, which may have been overlooked to date. Based on these findings, a strategy is proposed for the metabolic engineering of a future xylose-fermenting yeast for biofuel production.
[Mh] Termos MeSH primário: Frutose-Bifosfatase/metabolismo
[Mh] Termos MeSH secundário: Biocombustíveis/microbiologia
Biomassa
Etanol/metabolismo
Fermentação/fisiologia
Frutose/metabolismo
Glucose/metabolismo
Glicerol/metabolismo
Kluyveromyces/metabolismo
Kluyveromyces/fisiologia
Engenharia Metabólica/métodos
NAD/metabolismo
NADP/metabolismo
Via de Pentose Fosfato/fisiologia
Xilose/metabolismo
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Biofuels); 0U46U6E8UK (NAD); 30237-26-4 (Fructose); 3K9958V90M (Ethanol); 53-59-8 (NADP); A1TA934AKO (Xylose); EC 3.1.3.11 (Fructose-Bisphosphatase); IY9XDZ35W2 (Glucose); PDC6A3C0OX (Glycerol)
[Em] Mês de entrada:1709
[Cu] Atualização por classe:170911
[Lr] Data última revisão:
170911
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170526
[St] Status:MEDLINE
[do] DOI:10.1371/journal.pone.0177319


  8 / 2184 MEDLINE  
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[PMID]:28420223
[Au] Autor:Li N; Chang G; Xu Y; Ding Y; Li G; Yu T; Qing Y; Li J; Shen Y; Wang J; Wang X
[Ad] Endereço:Department of Medical Genetics and Molecular Diagnostic Laboratory, Shanghai Children's Medical Center, Shanghai Jiaotong University School of Medicine, Shanghai 200127, China. liniu0509@163.com.
[Ti] Título:Clinical and Molecular Characterization of Patients with Fructose 1,6-Bisphosphatase Deficiency.
[So] Source:Int J Mol Sci;18(4), 2017 Apr 18.
[Is] ISSN:1422-0067
[Cp] País de publicação:Switzerland
[La] Idioma:eng
[Ab] Resumo:Fructose-1,6-bisphosphatase (FBPase) deficiency is a rare, autosomal recessive inherited disease caused by the mutation of the gene, the incidence is estimated to be between 1/350,000 and 1/900,000. The symptoms of affected individuals are non-specific and are easily confused with other metabolic disorders. The present study describes the clinical features of four Chinese pediatric patients who presented with hypoglycemia, hyperlactacidemia, metabolic acidosis, and hyperuricemia. Targeted-next generation sequencing using the Agilent SureSelect XT Inherited Disease Panel was used to screen for causal variants in the genome, and the clinically-relevant variants were subsequently verified using Sanger sequencing. Here, DNA sequencing identified six variations of the gene (NM_000507.3) in the four patients. In Case 1, we found a compound heterozygous mutations of c.704delC (p.Pro235GlnfsX42) (novel) and c.960_961insG (p.Ser321Valfs) (known pathogenic). In Case 2, we found a compound heterozygous mutations of c.825 + 1G>A and c.960_961insG (both were known pathogenically). In Case 3, a homozygous missense mutation of c.355G>A (p.Asp119Asn) (reported in ClinVar database without functional study) was found. Case 4 had a compound heterozygous mutations c.720_729del (p.Tyr241GlyfsX33) (novel) and c.490G>A (p.Gly164Ser) (known pathogenically). Further in vitro studies in the COS-7cell line demonstrated that the mutation of ASP119ASN had no impact on protein expression, but decreased the enzyme activity, and with which the clinical significance of Asp119Asn can be determined to be likely pathogenic. This report not only expands upon the known spectrum of variation of the gene, but also deepens our understanding of the clinical features of FBPase deficiency.
[Mh] Termos MeSH primário: Deficiência de Frutose-1,6-Difosfatase/diagnóstico
Deficiência de Frutose-1,6-Difosfatase/genética
Frutose-Bifosfatase/genética
Mutação
Fenótipo
[Mh] Termos MeSH secundário: Idade de Início
Biomarcadores
Análise Química do Sangue
Encéfalo/diagnóstico por imagem
Encéfalo/patologia
Criança
Pré-Escolar
Análise Mutacional de DNA
Feminino
Frutose-Bifosfatase/metabolismo
Genótipo
Sequenciamento de Nucleotídeos em Larga Escala
Seres Humanos
Imagem por Ressonância Magnética
Masculino
Linhagem
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Biomarkers); EC 3.1.3.11 (Fructose-Bisphosphatase)
[Em] Mês de entrada:1706
[Cu] Atualização por classe:170629
[Lr] Data última revisão:
170629
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170420
[St] Status:MEDLINE


  9 / 2184 MEDLINE  
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[PMID]:28387640
[Au] Autor:Chen R; Li J; Zhou X; Liu J; Huang G
[Ad] Endereço:From the Department of Nuclear Medicine, Ren Ji Hospital, School of Medicine, Shanghai Jiao Tong University, 1630 Dongfang Rd, Shanghai 200127, China (R.C., J. Li, X.Z., J. Liu, G.H.); Department of Cancer Metabolism, Institute of Health Sciences, Chinese Academy of Sciences and Shanghai Jiao Tong U
[Ti] Título:Fructose-1,6-Bisphosphatase 1 Reduces F FDG Uptake in Hepatocellular Carcinoma.
[So] Source:Radiology;284(3):844-853, 2017 Sep.
[Is] ISSN:1527-1315
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:Purpose To determine whether fructose 1,6-bisphosphatase 1 (FBP1) expression is associated with fluorine 18 ( F) fluorodeoxyglucose (FDG) accumulation in patients with hepatocellular carcinoma (HCC) and to investigate how FBP1 expression and F FDG uptake are related to tumor differentiation grade and metabolism and whether the molecular mechanism involves hypoxia-inducible factor 1-α (HIF1A) transcriptional activity. Materials and Methods This retrospective study was approved by the institutional review board with informed consent. Eighty-five patients with HCC underwent F FDG combined positron emission tomography and computed tomography (PET/CT). The relationship between maximum standardized uptake (SUV ) and expression of FBP1, glucose transporter 1 (GLUT1), and hexokinase 2 (HK2) was analyzed with immunohistochemical analysis. In vitro FBP1 overexpression in HCC cells was used to examine the role of FBP1 in tumor metabolism, and its effect on HIF1A transcriptional activity was investigated with quantitative polymerase chain reaction and luciferase reporter assay. Spearman rank correlation was applied to determine the association between FBP1 expression and SUV . Results There was an inverse relationship between FBP1 expression and SUV (P = .003). SUV was higher in patients with poorly differentiated HCC (mean, 6.7 ± 3.6 [standard deviation]) than in those with well- (mean, 2.6 ± 0.7, P < .001) or moderately (mean, 4.1 ± 2.3, P < .001) differentiated HCC. FBP1 expression was significantly lower in patients with poorly differentiated HCC (mean, 0.6 ± 0.2) than in those with well- (mean, 1.4 ± 0.6, P = .006) or moderately (mean, 1.2 ± 0.2, P = .007) differentiated HCC. FBP1 overexpression in HCC cells led to a significant decrease in GLUT1 expression (P = .034), F FDG uptake (P = .023), and HIF1A transcriptional activity (P = .001). Conclusion SUV in patients with HCC is inversely associated with FBP1 expression, and FBP1 may inhibit F FDG uptake via the HIF1A pathway. SUV is higher in patients with poorly differentiated HCC than in those with well- or moderately differentiated HCC, which could be the result of lower FBP1 expression in the former. RSNA, 2017.
[Mh] Termos MeSH primário: Carcinoma Hepatocelular/enzimologia
Fluordesoxiglucose F18/farmacocinética
Frutose-Bifosfatase/metabolismo
Neoplasias Hepáticas/enzimologia
[Mh] Termos MeSH secundário: Adulto
Idoso
Carcinoma Hepatocelular/química
Carcinoma Hepatocelular/diagnóstico por imagem
Carcinoma Hepatocelular/patologia
Feminino
Frutose-Bifosfatase/análise
Células Hep G2
Seres Humanos
Imuno-Histoquímica
Neoplasias Hepáticas/química
Neoplasias Hepáticas/diagnóstico por imagem
Neoplasias Hepáticas/patologia
Masculino
Meia-Idade
Tomografia Computadorizada com Tomografia por Emissão de Pósitrons
Prognóstico
Estudos Retrospectivos
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0Z5B2CJX4D (Fluorodeoxyglucose F18); EC 3.1.3.11 (Fructose-Bisphosphatase)
[Em] Mês de entrada:1708
[Cu] Atualização por classe:170828
[Lr] Data última revisão:
170828
[Sb] Subgrupo de revista:AIM; IM
[Da] Data de entrada para processamento:170408
[St] Status:MEDLINE
[do] DOI:10.1148/radiol.2017161607


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[PMID]:28330423
[Au] Autor:Babukumar S; Vinothkumar V; Sankaranarayanan C; Srinivasan S
[Ad] Endereço:a Department of Biochemistry and Biotechnology, Faculty of Science , Annamalai University , Annamalainagar , India.
[Ti] Título:Geraniol, a natural monoterpene, ameliorates hyperglycemia by attenuating the key enzymes of carbohydrate metabolism in streptozotocin-induced diabetic rats.
[So] Source:Pharm Biol;55(1):1442-1449, 2017 Dec.
[Is] ISSN:1744-5116
[Cp] País de publicação:England
[La] Idioma:eng
[Ab] Resumo:CONTEXT: Geraniol, an acyclic monoterpene alcohol is found in medicinal plants, is used traditionally for several medical purposes including diabetes. OBJECTIVES: The present study evaluates the antihyperglycemic potential of geraniol on key enzymes of carbohydrate metabolism in streptozotocin (STZ)-induced diabetic rats. MATERIALS AND METHODS: Diabetes was induced in experimental rats, by a single intraperitoneal (i.p) injection of STZ [40 mg/kg body weight (b.w.)]. Different doses of geraniol (100, 200 and 400 mg/kg b.w.) and glyclazide (5 mg/kg b.w.) were administrated orally to diabetic rats for 45 days. Body weight, food intake, plasma glucose, insulin, blood haemoglobin (Hb), glycosylated haemoglobin (HbA ), hepatic glucose metabolic enzymes and glycogen were examined. RESULTS: The LD value of geraniol is 3600 mg/kg b.w. at oral administration in rats. Administration of geraniol in a dose-dependent manner (100, 200, 400 mg/kg b.w.) and glyclazide (5 mg/kg b.w.) for 45 days significantly improved the levels of insulin, Hb and decreased plasma glucose, HbA in diabetic-treated rats. Geraniol at its effective dose (200 mg/kg b.w.) ameliorated the altered activities of carbohydrate metabolic enzymes near normal effects compared with two other doses (100 and 400 mg/kg b.w.). Geraniol treatment to diabetic rats improved hepatic glycogen content suggesting its anti-hyperglycemic potential. Geraniol supplement was found to preserve the normal histological appearance of hepatic cells and pancreatic ß-cells in diabetic rats. DISCUSSION AND CONCLUSIONS: The present findings suggest that geraniol can potentially ameliorate key enzymes of glucose metabolism in experimental diabetes even though clinical studies used to evaluate this possibility are warranted.
[Mh] Termos MeSH primário: Glicemia/efeitos dos fármacos
Diabetes Mellitus Experimental/tratamento farmacológico
Hipoglicemiantes/farmacologia
Rim/efeitos dos fármacos
Fígado/efeitos dos fármacos
Estreptozocina
Terpenos/farmacologia
[Mh] Termos MeSH secundário: Animais
Biomarcadores/sangue
Glicemia/metabolismo
Diabetes Mellitus Experimental/sangue
Diabetes Mellitus Experimental/induzido quimicamente
Diabetes Mellitus Experimental/enzimologia
Relação Dose-Resposta a Droga
Frutose-Bifosfatase/metabolismo
Teste de Tolerância a Glucose
Glucose-6-Fosfatase/metabolismo
Glucosefosfato Desidrogenase/metabolismo
Hemoglobina A Glicada/metabolismo
Hexoquinase/metabolismo
Hipoglicemiantes/toxicidade
Insulina/sangue
Rim/enzimologia
Dose Letal Mediana
Fígado/enzimologia
Masculino
Pâncreas/efeitos dos fármacos
Pâncreas/patologia
Ratos Wistar
Terpenos/toxicidade
Fatores de Tempo
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Biomarkers); 0 (Blood Glucose); 0 (Glycated Hemoglobin A); 0 (Hypoglycemic Agents); 0 (Insulin); 0 (Terpenes); 5W494URQ81 (Streptozocin); EC 1.1.1.49 (Glucosephosphate Dehydrogenase); EC 2.7.1.1 (Hexokinase); EC 3.1.3.11 (Fructose-Bisphosphatase); EC 3.1.3.9 (Glucose-6-Phosphatase); L837108USY (geraniol)
[Em] Mês de entrada:1706
[Cu] Atualização por classe:171116
[Lr] Data última revisão:
171116
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170324
[St] Status:MEDLINE
[do] DOI:10.1080/13880209.2017.1301494



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