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Pesquisa : D05.750.078.280.300.300 [Categoria DeCS]
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[PMID]:29376591
[Au] Autor:Kasyan GR; Vishnevskii DA; Akulenko LV; Kozlova YO; Sharova EI; Tupikina NV; Pushkar' DY
[Ad] Endereço:Department of Urology, A.I. Evdokimov Moscow State University of Medicine and Dentistry, Moscow, Russia.
[Ti] Título:[Association of polymorphism of 1800255 COL3A1 gene with pelvic organ prolapse and urinary incontinence in women: preliminary data].
[So] Source:Urologiia;(6):30-33, 2017 Dec.
[Is] ISSN:1728-2985
[Cp] País de publicação:Russia (Federation)
[La] Idioma:rus
[Ab] Resumo:RELEVANCE: Collagen type I and III have a significant role in the development of pelvic organ prolapse (POP) and urinary incontinence in women. The role of the COL3A1 gene polymorphism remains debatable. Some studies and meta-analyzes have found a direct correlation between genetic defects and POP, while other researchers have not confirmed this association. This study aimed to investigate the association of the 1800255 COL3A1 gene polymorphism with the development of POP and urinary incontinence in women. MATERIALS AND METHODS: The study group comprised 52 patients (mean age 64.4 years) with verified POP and stress urinary incontinence. The control group included 21 patients without pelvic floor dysfunction. Patients were comparable in age and had at least one or more risk factors for developing pelvic floor dysfunction. Exclusion criteria for both groups were Marfan and Ehlers-Danlos syndromes and a history of surgery for POP or incontinence (for the control group). In all women, saliva samples were collected to detect polymorphism at the rs1800255 locus of the COL3A1 gene. Genotyping was conducted by Sanger sequencing. RESULTS: In patients with isolated genital prolapse, homozygous polymorphism (AA) had a low sensitivity (0.06) but an extremely high specificity (0.95). Heterozygote (GA) had the sensitivity of 0.35, the specificity of 0.53, and the AUC of 0.44. For urinary incontinence by homozygote (AA), sensitivity was 0.08, specificity 0.96, and by heterozygote (GA) 0.45 and 0.63, respectively. For the combination of pelvic prolapse and urinary incontinence by homozygote (AA), sensitivity was 0.07, specificity 1.0, and heterozygote (GA) 0.41 and 0.62, respectively. CONCLUSION: Given the high specificity of the polymorphism at the rs1800255 locus of the COL3A1 gene, determined by the Sanger sequencing, it can be concluded that there is an association between this polymorphism and urinary incontinence and POP in women.
[Mh] Termos MeSH primário: Colágeno Tipo III/genética
Prolapso de Órgão Pélvico/genética
Polimorfismo Genético
Incontinência Urinária/genética
[Mh] Termos MeSH secundário: Adulto
Idoso
Feminino
Seres Humanos
Meia-Idade
Prolapso de Órgão Pélvico/patologia
Prolapso de Órgão Pélvico/fisiopatologia
Incontinência Urinária/patologia
Incontinência Urinária/fisiopatologia
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (COL3A1 protein, human); 0 (Collagen Type III)
[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:180130
[St] Status:MEDLINE


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[PMID]:28453726
[Au] Autor:Li J; Yousefi K; Ding W; Singh J; Shehadeh LA
[Ad] Endereço:Department of Medicine, Division of Cardiology, University of Miami Leonard M. Miller School of Medicine, Miami, FL 33136, USA.
[Ti] Título:Osteopontin RNA aptamer can prevent and reverse pressure overload-induced heart failure.
[So] Source:Cardiovasc Res;113(6):633-643, 2017 May 01.
[Is] ISSN:1755-3245
[Cp] País de publicação:England
[La] Idioma:eng
[Ab] Resumo:Aims: Cardiac myocyte hypertrophy, the main compensatory response to chronic stress in the heart often progresses to a state of decompensation that can lead to heart failure. Osteopontin (OPN) is an effector for extracellular signalling that induces myocyte growth and fibrosis. Although increased OPN activity has been observed in stressed myocytes and fibroblasts, the detailed and long term effects of blocking OPN signalling on the heart remain poorly defined. Targeting cardiac OPN protein by an RNA aptamer may be beneficial for tuning down OPN pathologic signalling. We aimed to demonstrate the therapeutic effects of an OPN RNA aptamer on cardiac dysfunction. Methods and results: In vivo, we show that in a mouse model of pressure overload, treating at the time of surgeries with an OPN aptamer prevented cardiomyocyte hypertrophy and cardiac fibrosis, blocked OPN downstream signalling (PI3K and Akt phosphorylation), reduced expression of extracellular matrix (Lum, Col3a1, Fn1) and hypertrophy (Nppa, Nppb) genes, and prevented cardiac dysfunction. Treating at two months post-surgeries with the OPN aptamer reversed cardiac dysfunction and fibrosis and myocyte hypertrophy. While genetic homozygous deletion of OPN reduced myocardial wall thickness, surprisingly cardiac function and myocardial fibrosis, specifically collagen deposition and myofibroblast infiltration, were worse compared with wild type mice at three months of pressure overload. Conclusion: Taken together, these data demonstrate that tuning down cardiac OPN signalling by an OPN RNA aptamer is a novel and effective approach for preventing cardiac hypertrophy and fibrosis, improving cardiac function, and reversing pressure overload-induced heart failure.
[Mh] Termos MeSH primário: Aorta/fisiopatologia
Aptâmeros de Nucleotídeos/metabolismo
Pressão Arterial
Insuficiência Cardíaca/prevenção & controle
Hipertrofia Ventricular Esquerda/prevenção & controle
Miocárdio/metabolismo
Osteopontina/metabolismo
Disfunção Ventricular Esquerda/prevenção & controle
Função Ventricular Esquerda
Remodelação Ventricular
[Mh] Termos MeSH secundário: Animais
Aorta/cirurgia
Aptâmeros de Nucleotídeos/genética
Colágeno Tipo III/metabolismo
Citocinas/metabolismo
Modelos Animais de Doenças
Fibrose
Regulação da Expressão Gênica
Predisposição Genética para Doença
Insuficiência Cardíaca/genética
Insuficiência Cardíaca/metabolismo
Insuficiência Cardíaca/fisiopatologia
Hipertrofia Ventricular Esquerda/genética
Hipertrofia Ventricular Esquerda/metabolismo
Hipertrofia Ventricular Esquerda/fisiopatologia
Ligadura
Lumicana/metabolismo
Camundongos da Linhagem 129
Camundongos Endogâmicos C57BL
Camundongos Knockout
Miocárdio/patologia
Osteopontina/deficiência
Osteopontina/genética
Fenótipo
Fosfatidilinositol 3-Quinase/metabolismo
Fosforilação
Proteínas Proto-Oncogênicas c-akt/metabolismo
Transdução de Sinais
Fatores de Tempo
Disfunção Ventricular Esquerda/genética
Disfunção Ventricular Esquerda/metabolismo
Disfunção Ventricular Esquerda/fisiopatologia
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Aptamers, Nucleotide); 0 (COL3A1 protein, mouse); 0 (Collagen Type III); 0 (Cytokines); 0 (Lum protein, mouse); 0 (Lumican); 0 (Spp1 protein, mouse); 106441-73-0 (Osteopontin); EC 2.7.1.137 (Phosphatidylinositol 3-Kinase); EC 2.7.11.1 (Proto-Oncogene Proteins c-akt)
[Em] Mês de entrada:1802
[Cu] Atualização por classe:180308
[Lr] Data última revisão:
180308
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170429
[St] Status:MEDLINE
[do] DOI:10.1093/cvr/cvx016


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[PMID]:29216800
[Au] Autor:Gu G; Yang H; Cui L; Fu Y; Li F; Zhou Z; Zheng Y
[Ad] Endereço:1 Department of Vascular Surgery, Peking Union Medical College Hospital, Beijing, China.
[Ti] Título:Vascular Ehlers-Danlos Syndrome With a Novel Missense COL3A1 Mutation Present With Pulmonary Complications and Iliac Arterial Dissection.
[So] Source:Vasc Endovascular Surg;52(2):138-142, 2018 Feb.
[Is] ISSN:1938-9116
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:Vascular Ehlers-Danlos syndrome (vEDS) is a life-threatening connective tissue disorder due to its high tendency of arterial and organ rupture. Pulmonary complications in vEDS are rare. We present a young male patient with vEDS who developed severe pulmonary complications and severe rupture of the iliac artery at different stages of his life. Vascular Ehlers-Danlos syndrome was diagnosed based on clinical manifestations and confirmed by the identification of COL3A1 gene mutation. Due to high bleeding tendency and weak cardiopulmonary capacity, conservative treatment was taken for him. To our knowledge, this is the first report of vEDS case in which the patient developed both pulmonary complications and dissection of large arteries. Our report emphasizes the importance of considering vEDS when an adolescent develops unexplained pulmonary cysts with fragility of lung tissues. Genetic counseling and close monitoring should be performed for earlier diagnosis and prevention of severe complications of large arteries. The typical presentations of vEDS were also discussed by means of a review of case reports on vEDS with pulmonary complications.
[Mh] Termos MeSH primário: Aneurisma Roto/etiologia
Colágeno Tipo III/genética
Síndrome de Ehlers-Danlos/genética
Aneurisma Ilíaco/etiologia
Pneumopatias/etiologia
Mutação de Sentido Incorreto
[Mh] Termos MeSH secundário: Adolescente
Aneurisma Roto/diagnóstico por imagem
Aneurisma Roto/terapia
Angiografia por Tomografia Computadorizada
Análise Mutacional de DNA
Síndrome de Ehlers-Danlos/complicações
Síndrome de Ehlers-Danlos/diagnóstico
Predisposição Genética para Doença
Seres Humanos
Aneurisma Ilíaco/diagnóstico por imagem
Pneumopatias/diagnóstico por imagem
Pneumopatias/cirurgia
Masculino
Fenótipo
Pneumonectomia
Resultado do Tratamento
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE; REVIEW
[Nm] Nome de substância:
0 (COL3A1 protein, human); 0 (Collagen Type III)
[Em] Mês de entrada:1802
[Cu] Atualização por classe:180228
[Lr] Data última revisão:
180228
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:171209
[St] Status:MEDLINE
[do] DOI:10.1177/1538574417745432


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[PMID]:28460256
[Au] Autor:Rubis P; Wisniowska-Smialek S; Wypasek E; Rudnicka-Sosin L; Hlawaty M; Lesniak-Sobelga A; Kostkiewicz M; Podolec P
[Ad] Endereço:Department of Cardiac and Vascular Diseases, John Paul II Hospital, 31-202 Krakow, Pradnicka Street 80, Poland. Electronic address: pawelrub@poczta.onet.pl.
[Ti] Título:12-month patterns of serum markers of collagen synthesis, transforming growth factor and connective tissue growth factor are similar in new-onset and chronic dilated cardiomyopathy in patients both with and without cardiac fibrosis.
[So] Source:Cytokine;96:217-227, 2017 08.
[Is] ISSN:1096-0023
[Cp] País de publicação:England
[La] Idioma:eng
[Ab] Resumo:BACKGROUND: The dynamics of the extracellular matrix (ECM) fibrosis process in dilated cardiomyopathy (DCM) may be assessed non-invasively by means of serum markers of fibrosis. AIM: To explore the kinetics of serum markers of fibrosis during a 12-month follow-up in DCM. METHODS: We included 70 consecutive DCM patients (pts) (48±12.1years, EF 24.4±7.4%) with new-onset (n=35, duration <6months) and chronic DCM (n=35, >6months). Markers of collagen type I and III synthesis - procollagens type I and III carboxy- and amino-terminal peptides (PICP, PINP, PIIICP, PIIINP), and ECM metabolism controlling factors - tumor growth factor beta-1 (TGF1-ß), and connective tissue growth factor (CTGF) - were measured in serum at baseline, and at 3- and 12-month follow-up. All pts underwent endomyocardial biopsy to determine the presence and extent of ECM fibrosis. RESULTS: Markers of collagen type I synthesis (PICP and PINP) were almost homogenously increased over the 3- and 12-month period, whereas PIIINP values decreased and PIIICP levels were unchanged in new-onset and chronic DCM, and in pts with and without ECM fibrosis. Both TGF-ß and CTGF levels decreased over the observation period. Kinetics of serum markers of collagen synthesis and fibrosis controlling factors did not differ between DCM pts categorized according to disease duration and fibrosis status. CONCLUSIONS: The kinetics of collagen type I and III synthesis in DCM move in opposite directions, with production of collagen type I consistently increasing, and the synthesis of collagen type III decreasing. Levels of TGF and CTGF, which are proven fibrosis-stimulating factors, had a tendency to decrease. Regardless of disease duration or fibrosis status, the kinetics of serum markers of collagen synthesis, TGF and CTGF were similar in DCM. A better understanding of the kinetics of serum markers of fibrosis in DCM may help to develop more tailored therapeutic approaches to fibrosis.
[Mh] Termos MeSH primário: Cardiomiopatia Dilatada/sangue
Colágeno Tipo III/sangue
Colágeno Tipo I/sangue
Fator de Crescimento do Tecido Conjuntivo/sangue
Fibrose Endomiocárdica/sangue
Fibrose/sangue
Fatores de Crescimento Transformadores/sangue
[Mh] Termos MeSH secundário: Adulto
Biomarcadores/sangue
Cardiomiopatia Dilatada/complicações
Colágeno Tipo I/biossíntese
Colágeno Tipo III/biossíntese
Fibrose Endomiocárdica/complicações
Feminino
Fibrose/terapia
Seguimentos
Seres Humanos
Cinética
Masculino
Meia-Idade
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Biomarkers); 0 (Collagen Type I); 0 (Collagen Type III); 139568-91-5 (Connective Tissue Growth Factor); 76057-06-2 (Transforming Growth Factors)
[Em] Mês de entrada:1802
[Cu] Atualização por classe:180227
[Lr] Data última revisão:
180227
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:170502
[St] Status:MEDLINE


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[PMID]:29254291
[Au] Autor:Omar NN; El-Tawdi AH; Tash RF; Shoukry Y; Mahmoud NA; El Bakly W
[Ad] Endereço:Department of Biochemistry, Faculty of Pharmacy, Modern University for Technology and Information, Cairo, Egypt.
[Ti] Título:Tumor potential in rat wounds after short- and long-term administration of platelet-rich plasma.
[So] Source:J Biol Regul Homeost Agents;31(4):889-899, 2017 Oct-Dec.
[Is] ISSN:0393-974X
[Cp] País de publicação:Italy
[La] Idioma:eng
[Ab] Resumo:Platelet-rich plasma (PRP) has been recognized as an effective strategy for tissue regeneration, how-ever, the safety of PRP in wound healing in terms of tumorigenicity has not yet been addressed. Therefore, the aim of this study was to examine the impact of PRP administration on the expression of the inflammatory marker, tenascin-C (TnC) and the myofibroblast markers, α-smooth muscle actin (α-SMA) and vimentin. The immune suppressive response was examined by determining the level of forkhead box protein 3 (Foxp3). PRP was administered for both long-term (two times weekly for four weeks) and short-term (for the fourth week only) post-wounding. Collagen I (col1) and lysyl oxidase (LOX) were used to indicate complete healing, after which any increase in the myofibroblast or in the inflammatory markers would suggest tumor potential. Collagen III (col3), a marker for granulation tissue, was used to remark non-healing. Quantitative real-time reverse transcriptase polymerase chain reaction (QRT-PCR) and Western blot showed that after long-term administration of PRP, the expression of TnC, α-SMA and vimentin was barely detected, while being markedly expressed in the wounded non-treated group and in the short-term administration group. Moreover, the active expression of α-SMA in the two groups was associated positively with the expression of col3 and negatively with the expression of col1. The low expression of Foxp3 after short-term administration relative to the control group indicated active immunity against tumor development. In conclusion, these findings indicate that PRP can be safely used in short- and long-term administration without tumorigenesis concern.
[Mh] Termos MeSH primário: Plasma Rico em Plaquetas/fisiologia
Ferida Cirúrgica/terapia
Cicatrização/fisiologia
[Mh] Termos MeSH secundário: Actinas/genética
Actinas/metabolismo
Animais
Biomarcadores/metabolismo
Carcinogênese
Colágeno Tipo I/genética
Colágeno Tipo I/metabolismo
Colágeno Tipo III/genética
Colágeno Tipo III/metabolismo
Feminino
Fatores de Transcrição Forkhead/genética
Fatores de Transcrição Forkhead/metabolismo
Expressão Gênica
Miofibroblastos/metabolismo
Miofibroblastos/patologia
Ratos
Ratos Sprague-Dawley
Ferida Cirúrgica/genética
Ferida Cirúrgica/metabolismo
Ferida Cirúrgica/patologia
Tenascina/genética
Tenascina/metabolismo
Vimentina/genética
Vimentina/metabolismo
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Actins); 0 (Biomarkers); 0 (Collagen Type I); 0 (Collagen Type III); 0 (Forkhead Transcription Factors); 0 (Foxp3 protein, rat); 0 (Tenascin); 0 (Vimentin); 0 (smooth muscle actin, rat)
[Em] Mês de entrada:1802
[Cu] Atualização por classe:180222
[Lr] Data última revisão:
180222
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:171220
[St] Status:MEDLINE


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[PMID]:29249634
[Au] Autor:Hirose M; Takano H; Hasegawa H; Tadokoro H; Hashimoto N; Takemura G; Kobayashi Y
[Ad] Endereço:Department of Cardiovascular Medicine, Chiba University Graduate School of Medicine, Chiba 260-8670, Japan.
[Ti] Título:The effects of dipeptidyl peptidase-4 on cardiac fibrosis in pressure overload-induced heart failure.
[So] Source:J Pharmacol Sci;135(4):164-173, 2017 Dec.
[Is] ISSN:1347-8648
[Cp] País de publicação:Japan
[La] Idioma:eng
[Ab] Resumo:Dipeptidyl peptidase-4 (DPP-4) inhibitors are hypoglycemic agents. DPP-4 inhibitor has cardioprotective effects after transverse aortic constriction (TAC), but role of DPP-4 on cardiac fibrosis after TAC is not well known. Our aim was to determine the effects of DPP-4 on cardiac fibrosis in murine TAC model. Wild-type mice and DPP-4 knockout mice were subjected to TAC. Wild-type mice were then treated with vehicle or DPP-4 inhibitor. DPP-4 activities in serum and heart tissue were significantly increased at 2 weeks after TAC, but they were significantly decreased by DPP-4 inhibitor treatment. The inhibition of DPP-4 did not affect left ventricular hypertrophy, but improved cardiac function and decreased myocardial and perivascular fibrosis after TAC. The inhibition of DPP-4 decreased the collagen type III/I ratio in myocardium. These results suggest that DPP-4 inhibition ameliorates the progression of heart failure after TAC by changing the quality and quantity of cardiac fibrosis.
[Mh] Termos MeSH primário: Cardiotônicos
Dipeptidil Peptidase 4/fisiologia
Inibidores da Dipeptidil Peptidase IV/farmacologia
Inibidores da Dipeptidil Peptidase IV/uso terapêutico
Insuficiência Cardíaca/tratamento farmacológico
Insuficiência Cardíaca/etiologia
Miocárdio/patologia
[Mh] Termos MeSH secundário: Animais
Aorta
Estenose da Valva Aórtica/complicações
Colágeno Tipo I/metabolismo
Colágeno Tipo III/metabolismo
Constrição Patológica
Dipeptidil Peptidase 4/metabolismo
Modelos Animais de Doenças
Fibrose
Insuficiência Cardíaca/patologia
Hipertensão/complicações
Hipertrofia
Masculino
Camundongos Endogâmicos C57BL
Miocárdio/metabolismo
Pressão
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Cardiotonic Agents); 0 (Collagen Type I); 0 (Collagen Type III); 0 (Dipeptidyl-Peptidase IV Inhibitors); EC 3.4.14.5 (Dipeptidyl Peptidase 4)
[Em] Mês de entrada:1802
[Cu] Atualização por classe:180222
[Lr] Data última revisão:
180222
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:171219
[St] Status:MEDLINE


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[PMID]:29310440
[Au] Autor:Kodama H; Kumai Y; Nishimoto K; Toya Y; Miyamaru S; Furushima S; Yumoto E
[Ad] Endereço:1 Department of Otolaryngology Head and Neck Surgery, Kumamoto University Graduate School of Medicine, Kumamoto, Japan.
[Ti] Título:The Ferret as a Surgical Model for Vocal Fold Scar Creation and Treatment.
[So] Source:Ann Otol Rhinol Laryngol;127(3):146-154, 2018 Mar.
[Is] ISSN:1943-572X
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:OBJECTIVES: To develop a vocal fold (VF) scarring procedure in the ferret, characterize the scars histologically, and test the injectability of the lamina propria (LP). Secondarily, to compare laryngeal anatomy of the ferret with rat and rabbit. MATERIALS AND METHODS: The larynges of 18 male ferrets were prepared by unilateral scarring, and normal larynges from 6 female Wistar rats and 5 male albino rabbits were used for comparative purposes. For scarring, the right VF were electrocauterized, ablating the entire LP. Prior to harvesting the larynges at 4 and 16 weeks, each ferret was re-anesthetized, and in 3 animals, India ink was injected into the LPs of both normal and scarred VFs. RESULTS: Laryngoscopic methods and instrumentation for precise visualization, scarring, and injection were developed. The scarred VFs had reduced hyaluronic acid and increased collagen type I, III, and fibronectin compared with normal VFs. The 2 timepoints (4 and 16 weeks) differed significantly only in collagen type III level (levels were higher at 4 weeks). Injected ink migrated from scarred LP to muscle layer just beneath the scarred tissue 3 hours after injection. CONCLUSION: The ferret is a promising species for creation and experimental treatment of vocal fold scar.
[Mh] Termos MeSH primário: Cicatriz
Eletrocoagulação/métodos
Laringoscopia
Membrana Mucosa
Prega Vocal/cirurgia
[Mh] Termos MeSH secundário: Animais
Cicatriz/etiologia
Cicatriz/metabolismo
Cicatriz/patologia
Colágeno Tipo I/análise
Colágeno Tipo III/análise
Feminino
Furões
Fibronectinas/análise
Laringoscopia/instrumentação
Laringoscopia/métodos
Modelos Anatômicos
Membrana Mucosa/patologia
Membrana Mucosa/cirurgia
Coelhos
Ratos
Prega Vocal/patologia
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (Collagen Type I); 0 (Collagen Type III); 0 (Fibronectins)
[Em] Mês de entrada:1802
[Cu] Atualização por classe:180221
[Lr] Data última revisão:
180221
[Sb] Subgrupo de revista:AIM; IM
[Da] Data de entrada para processamento:180110
[St] Status:MEDLINE
[do] DOI:10.1177/0003489417750165


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[PMID]:29346445
[Au] Autor:Chiarelli N; Carini G; Zoppi N; Ritelli M; Colombi M
[Ad] Endereço:Department of Molecular and Translational Medicine, Division of Biology and Genetics, University of Brescia, Brescia, Italy.
[Ti] Título:Transcriptome analysis of skin fibroblasts with dominant negative COL3A1 mutations provides molecular insights into the etiopathology of vascular Ehlers-Danlos syndrome.
[So] Source:PLoS One;13(1):e0191220, 2018.
[Is] ISSN:1932-6203
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:Vascular Ehlers-Danlos syndrome (vEDS) is a dominantly inherited connective tissue disorder caused by mutations in the COL3A1 gene that encodes type III collagen (COLLIII), which is the major expressed collagen in blood vessels and hollow organs. The majority of disease-causing variants in COL3A1 are glycine substitutions and in-frame splice mutations in the triple helix domain that through a dominant negative effect are associated with the severe clinical spectrum potentially lethal of vEDS, characterized by fragility of soft connective tissues with arterial and organ ruptures. To shed lights into molecular mechanisms underlying vEDS, we performed gene expression profiling in cultured skin fibroblasts from three patients with different structural COL3A1 mutations. Transcriptome analysis revealed significant changes in the expression levels of several genes involved in maintenance of cell redox and endoplasmic reticulum (ER) homeostasis, COLLs folding and extracellular matrix (ECM) organization, formation of the proteasome complex, and cell cycle regulation. Protein analyses showed that aberrant COLLIII expression is associated with the disassembly of many structural ECM constituents, such as fibrillins, EMILINs, and elastin, as well as with the reduction of the proteoglycans perlecan, decorin, and versican, all playing an important role in the vascular system. Furthermore, the altered distribution of the ER marker protein disulfide isomerase PDI and the strong reduction of the COLLs-modifying enzyme FKBP22 are consistent with the disturbance of ER-related homeostasis and COLLs biosynthesis and post-translational modifications, indicated by microarray analysis. Our findings add new insights into the pathophysiology of this severe vascular disorder, since they provide a picture of the gene expression changes in vEDS skin fibroblasts and highlight that dominant negative mutations in COL3A1 also affect post-translational modifications and deposition into the ECM of several structural proteins crucial to the integrity of soft connective tissues.
[Mh] Termos MeSH primário: Colágeno Tipo III/genética
Síndrome de Ehlers-Danlos/genética
Mutação
[Mh] Termos MeSH secundário: Substituição de Aminoácidos
Ciclo Celular/genética
Células Cultivadas
Colágeno Tipo III/química
Colágeno Tipo III/metabolismo
Síndrome de Ehlers-Danlos/etiologia
Síndrome de Ehlers-Danlos/metabolismo
Matriz Extracelular/metabolismo
Fibroblastos/metabolismo
Perfilação da Expressão Gênica
Seres Humanos
Proteínas Mutantes/química
Proteínas Mutantes/genética
Proteínas Mutantes/metabolismo
Mutação de Sentido Incorreto
Dobramento de Proteína
Processamento de Proteína Pós-Traducional
Pele/metabolismo
[Pt] Tipo de publicação:JOURNAL ARTICLE
[Nm] Nome de substância:
0 (COL3A1 protein, human); 0 (Collagen Type III); 0 (Mutant Proteins)
[Em] Mês de entrada:1802
[Cu] Atualização por classe:180220
[Lr] Data última revisão:
180220
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:180119
[St] Status:MEDLINE
[do] DOI:10.1371/journal.pone.0191220


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[PMID]:29337054
[Au] Autor:Han SJ; Jung JK; Im SS; Lee SR; Jang BC; Park KM; Kim JI
[Ad] Endereço:Department of Anatomy and BK21 Plus, Kyungpook National University School of Medicine, Daegu, 700-422, Republic of Korea.
[Ti] Título:Deficiency of primary cilia in kidney epithelial cells induces epithelial to mesenchymal transition.
[So] Source:Biochem Biophys Res Commun;496(2):450-454, 2018 02 05.
[Is] ISSN:1090-2104
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:Primary cilium is a microtubule-based non-motile organelle that plays critical roles in kidney pathophysiology. Our previous studies revealed that the lengths of primary cilia decreased upon renal ischemia/reperfusion injury and oxidative stress, and restored with recovery. Here, we tested the hypothesis that lack of primary cilium causes epithelial to mesenchymal transition (EMT) of kidney tubule cells. We investigated the alteration of length of primary cilia in TGF-ß-induced EMT via visualization of primary cilia by fluorescence staining against acetylated α-tubulin. EMT was determined by measuring mesenchymal protein expression using quantitative PCR and indirect fluorescence staining. As a result, TGF-ß treatment decreased ciliary length along with EMT. To test whether defect of primary cilia trigger onset of EMT, cilia formation was disturbed by knock down of ciliary protein using siRNA along with/without TGF-ß treatment. Knock down of Arl13b and Ift20 reduced cilia elongation and increased expression of EMT markers such as fibronectin, α-SMA, and collagen III. TGF-ß-induced EMT was greater as well in Arl13b and Ift20-knock down cells compared to control cells. Taken together, deficiency of primary cilia trigger EMT and exacerbates it under pro-fibrotic signals.
[Mh] Termos MeSH primário: Cílios/efeitos dos fármacos
Transição Epitelial-Mesenquimal/efeitos dos fármacos
Fator de Crescimento Transformador beta/farmacologia
Tubulina (Proteína)/genética
[Mh] Termos MeSH secundário: Fatores de Ribosilação do ADP/antagonistas & inibidores
Fatores de Ribosilação do ADP/genética
Fatores de Ribosilação do ADP/metabolismo
Actinas/genética
Actinas/metabolismo
Animais
Proteínas de Transporte/antagonistas & inibidores
Proteínas de Transporte/genética
Proteínas de Transporte/metabolismo
Tamanho Celular
Cílios/metabolismo
Cílios/ultraestrutura
Colágeno Tipo III/genética
Colágeno Tipo III/metabolismo
Cães
Transição Epitelial-Mesenquimal/genética
Fibronectinas/genética
Fibronectinas/metabolismo
Regulação da Expressão Gênica
Células Madin Darby de Rim Canino
RNA Interferente Pequeno/genética
RNA Interferente Pequeno/metabolismo
Transdução de Sinais
Tubulina (Proteína)/metabolismo
[Pt] Tipo de publicação:JOURNAL ARTICLE; RESEARCH SUPPORT, NON-U.S. GOV'T
[Nm] Nome de substância:
0 (Actins); 0 (Carrier Proteins); 0 (Collagen Type III); 0 (Fibronectins); 0 (RNA, Small Interfering); 0 (Transforming Growth Factor beta); 0 (Tubulin); EC 3.6.5.2 (ADP-Ribosylation Factors)
[Em] Mês de entrada:1802
[Cu] Atualização por classe:180213
[Lr] Data última revisão:
180213
[Sb] Subgrupo de revista:IM
[Da] Data de entrada para processamento:180117
[St] Status:MEDLINE


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[PMID]:29381997
[Au] Autor:Park MA; Shin SY; Kim YJ; Park MJ; Lee SH
[Ad] Endereço:Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Kyung Hee University School of Medicine.
[Ti] Título:Vascular Ehlers-Danlos syndrome with cryptorchidism, recurrent pneumothorax, and pulmonary capillary hemangiomatosis-like foci: A case report.
[So] Source:Medicine (Baltimore);96(47):e8853, 2017 Nov.
[Is] ISSN:1536-5964
[Cp] País de publicação:United States
[La] Idioma:eng
[Ab] Resumo:RATIONALE: Vascular Ehlers-Danlos syndrome (vEDS) is a rare autosomal dominant inherited collagen disorder caused by defects or deficiency of pro-alpha 1 chain of type III procollagen encoded by COL3A1. vEDS is characterized not only by soft tissue manifestations including hyperextensibility of skin and joint hypermobility but also by early mortality due to rupture of arteries or vital organs. Although pulmonary complications are not common, vEDS cases complicated by pneumothorax, hemothorax, or intrapulmonary hematoma have been reported. When a patient initially presents only with pulmonary complications, it is not easy for clinicians to suspect vEDS. PATIENT CONCERNS: We report a case of an 18-year-old high school student, with a past history of cryptorchidism, presenting with recurrent pneumothorax. DIAGNOSES: Routine laboratory findings were unremarkable. Chest high resolution computed tomographic scan showed age-unmatched hyperinflation of both lungs, atypical cystic changes and multifocal ground glass opacities scattered in both lower lobes. His slender body shape, hyperflexible joints, and hyperextensible skin provided clue to suspicion of a possible connective tissue disorder. INTERVENTIONS: The histological examination of the lung lesions showed excessive capillary proliferation in the pulmonary interstitium and pleura allowing the diagnosis of pulmonary capillary hemangiomatosis (PCH)-like foci. Genetic study revealed COL3A1 gene splicing site mutation confirming his diagnosis as vEDS. OUTCOMES: Although his diagnosis vEDS is notorious for fatal vascular complication, there was no evidence of such complication at presentation. Fortunately, he has been followed up for 10 months without pulmonary or vascular complications. LESSONS: To the best of our knowledge, both cryptorchidism and PCH-like foci have never been reported yet as complications of vEDS, suggesting our case might be a new variant of this condition. This case emphasizes the importance of comprehensive physical examination and history-taking, and the clinical suspicion of a possible connective tissue disorder when we encounter cases with atypical presentation and/or unique chest radiologic findings especially in young patients.
[Mh] Termos MeSH primário: Criptorquidismo/etiologia
Síndrome de Ehlers-Danlos/complicações
Hemangioma Capilar/etiologia
Hipertensão Pulmonar/etiologia
Neoplasias Pulmonares/etiologia
Pneumotórax/etiologia
[Mh] Termos MeSH secundário: Adolescente
Colágeno Tipo III/análise
Síndrome de Ehlers-Danlos/diagnóstico
Síndrome de Ehlers-Danlos/genética
Seres Humanos
Masculino
Recidiva
[Pt] Tipo de publicação:CASE REPORTS; JOURNAL ARTICLE
[Nm] Nome de substância:
0 (COL3A1 protein, human); 0 (Collagen Type III)
[Em] Mês de entrada:1802
[Cu] Atualização por classe:180209
[Lr] Data última revisão:
180209
[Sb] Subgrupo de revista:AIM; IM
[Da] Data de entrada para processamento:180201
[St] Status:MEDLINE
[do] DOI:10.1097/MD.0000000000008853



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