Chaperones
Mostrando 1-12 de 545 artigos, teses e dissertações.
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1. Fabry disease: genetics, pathology, and treatment
SUMMARY Fabry disease (FD) is a recessive monogenic inheritance disease linked to chromosome X, secondary to mutations in the GLA gene. Its prevalence is estimated between 1:8,454 and 1:117,000 among males and is probably underdiagnosed. Mutations in the GLA gene lead to the progressive accumulation of globotriaosylceramide (Gb3). Gb3 accumulates in lysosome
Rev. Assoc. Med. Bras.. Publicado em: 13/01/2020
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2. Small Molecules: Substrate Inhibitors, Chaperones, Stop-Codon Read Through, and Beyond
Abstract Lysosomal storage disorders are rare genetic disorders due to deficient lysosomal activity, which leads to progressive accumulation of nonmetabolized substrates. Patient’s clinical outcomes have significantly improved since the advent of enzyme replacement therapy, even though this therapeutic approach presents important limitations, such as immun
J. inborn errors metab. screen.. Publicado em: 30/05/2019
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3. Enzyme Replacement Therapy for Fabry Disease
Abstract Fabry disease is a rare X-linked disease caused by the deficiency of α-galactosidase that leads to the accumulation of abnormal glycolipid. Untreated patients develop potentially lethal complications by age 30 to 50 years. Enzyme replacement therapy is the current standard of therapy for Fabry disease. Two formulations of recombinant human α-galac
J. inborn errors metab. screen.. Publicado em: 30/05/2019
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4. Heterologous Expression of a Putative ClpC Chaperone Gene Leads to Induction of a Host Metabolite
Genome mining provides exciting opportunities for the discovery of natural products. However, in contrast to traditional bioassay-guided approaches, challenges of genome mining include poor or no expression of biosynthetic gene clusters (BGCs). Additionally, given that thousands of BGCs are now available through extensive genome sequencing, how does one sele
J. Braz. Chem. Soc.. Publicado em: 2019-03
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5. α-Linolenic acid prevents hepatic steatosis and improves glucose tolerance in mice fed a high-fat diet
OBJECTIVES: Dietary omega-3 fatty acids have been efficacious in decreasing serum cholesterol levels and reducing the risk of cardiovascular disease. However, the metabolic and molecular changes induced by the omega-3 fatty acid α-linolenic acid (ALA), which is found in linseed oil, are not fully understood. In this study, we showed a correlation between A
Clinics. Publicado em: 29/10/2018
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6. Heat Shock Proteins: Protection and Potential Biomarkers for Ischemic Injury of Cardiomyocytes After Surgery
Abstract The heat shock proteins are endogenous proteins with the ability to act as molecular chaperones. Methods that provide cell protection by way of some damage can positively influence the results of surgery. The present review summarizes current knowledge concerning the cardioprotective role of the heat shock proteins as occurs in heart damage, includi
Braz. J. Cardiovasc. Surg.. Publicado em: 2018-06
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7. Immunoproteomics of Plasmodium falciparum-infected red blood cell membrane fractions
BACKGROUND The surface of infected red blood cells (iRBCs) has been widely investigated because of the molecular complexity and pathogenesis mechanisms involved. Asymptomatic individuals are important in the field because they can perpetuate transmission as natural reservoirs and present a challenge for diagnosing malaria because of their low levels of circ
Mem. Inst. Oswaldo Cruz. Publicado em: 2017-12
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8. The F309S mutation increases factor VIII secretion in human cell line
ABSTRACT OBJECTIVES: The capacity of a human cell line to secrete recombinant factor VIII with a F309S point mutation was investigated, as was the effect of the addition of chemical chaperones (betaine and sodium-4-phenylbutyrate) on the secretion of factor VIII. METHODS: This work used a vector with a F309S mutation in the A1 domain to investigate FVIII
Rev. Bras. Hematol. Hemoter.. Publicado em: 2016-06
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9. Proteomic analysis of halotolerant proteins under high and low salt stress in Dunaliella salina using two-dimensional differential in-gel electrophoresis
Abstract Dunaliella salina, a single-celled marine alga with extreme salt tolerance, is an important model organism for studying fundamental extremophile survival mechanisms and their potential practical applications. In this study, two-dimensional differential in-gel electrophoresis (2D-DIGE) was used to investigate the expression of halotolerant proteins u
Genet. Mol. Biol.. Publicado em: 13/05/2016
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10. Disaggregases, molecular chaperones that resolubilize protein aggregates
O processo de enovelamento é um evento importante para o tempo de vida de uma proteína, uma vez que é essencial para a função adequada da proteína e, por conseguinte, para a fisiologia celular. Enovelamento inadequado, ou mau enovelamento, pode não só conduzir à perda de função, mas também para a formação de agregados de proteína, uma associa�
An. Acad. Bras. Ciênc.. Publicado em: 25/08/2015
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11. Characteristics of dr1790 disruptant and its functional analysis in Deinococcus radiodurans
Deinococcus radiodurans (DR) is an extremophile that is well known for its resistance to radiation, oxidants and desiccation. The gene dr1790 of D. radiodurans was predicted to encode a yellow-related protein. The primary objective of the present study was to characterize the biological function of the DR1790 protein, which is a member of the ancient yellow/
Braz. J. Microbiol.. Publicado em: 2015-06
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12. Different levels of hsp70 and hsc70 mRNA expression in Iberian fish exposed to distinct river conditions
Comprehension of the mechanisms by which ectotherms, such as fish, respond to thermal stress is paramount for understanding the threats that environmental changes may pose to wild populations. Heat shock proteins are molecular chaperones with an important role in several stress conditions such as high temperatures. In the Iberian Peninsula, particularly in P
Genet. Mol. Biol.. Publicado em: 2013