Abstract:
:Oxidative stress and an increase in the levels of free radicals are important markers associated with several pathologies, including Alzheimer's disease, cancer and diabetes. Friedreich's ataxia (FRDA) is an excellent paradigmatic example of a disease in which oxidative stress plays an important, albeit incompletely understood, role. FRDA is a rare genetic neurodegenerative disease that involves the partial silencing of frataxin, a small mitochondrial protein that was completely overlooked before being linked to FRDA. More than 20 years later, we now know how important this protein is in terms of being an essential and vital part of the machinery that produces iron-sulfur clusters in the cell. In this review, we revisit the most important steps that have brought us to our current understanding of the function of frataxin and its role in disease. We discuss the current hypotheses on the role of oxidative stress in FRDA and review some of the existing animal and cellular models. We also evaluate new techniques that can assist in the study of the disease mechanisms, as well as in our understanding of the interplay between primary and secondary phenotypes.
journal_name
FEBS Lettjournal_title
FEBS lettersauthors
Lupoli F,Vannocci T,Longo G,Niccolai N,Pastore Adoi
10.1002/1873-3468.12928subject
Has Abstractpub_date
2018-03-01 00:00:00pages
718-727issue
5eissn
0014-5793issn
1873-3468journal_volume
592pub_type
杂志文章,评审相关文献
FEBS LETTERS文献大全abstract::Single rat hepatocytes microinjected with aequorin show free Ca oscillations when stimulated with Ca(2+)-mobilizing hormones. We show here that an inhibitor of diacylglycerol kinase (R 59 022) and an analogue of native diacylglycerol (diC8) inhibit free Ca oscillations induced by phenylephrine and vasopressin. These r...
journal_title:FEBS letters
pub_type: 杂志文章
doi:10.1016/0014-5793(92)81409-f
更新日期:1992-11-02 00:00:00
abstract::VinJ is an amidohydrolase belonging to the serine peptidase family that catalyzes the hydrolysis of the terminal aminoacyl moiety of a polyketide intermediate during the biosynthesis of vicenistatin. Herein, we report the crystal structure of VinJ. VinJ possesses a unique hydrophobic tunnel for the recognition of the ...
journal_title:FEBS letters
pub_type: 杂志文章
doi:10.1016/j.febslet.2014.01.060
更新日期:2014-03-18 00:00:00
abstract::Muscle phosphofructokinase interacts with microtubule-associated protein-free microtubules resulting in a reduction of the overall activity of the enzyme [Lehotzky et al. (1993) J. Biol. Chem. 268, 10888-10894] and periodical cross-linking of the tubules [Lehotzky et al. (1994) Biochem. Biophys. Res. Commun. 204, 585-...
journal_title:FEBS letters
pub_type: 杂志文章
doi:10.1016/0014-5793(95)01510-8
更新日期:1996-01-29 00:00:00
abstract::Zona pellucida (ZP) binding proteins from boar spermatozoa were compared with antigens recognized by ACR.2 and ACR.3 monoclonal antibodies. The ZP binding proteins of 55, 53, 45 and 38 kDa are identical with various forms of boar acrosin immunologically detected by ACR.2 antibody. The ZP binding proteins of 17 and 18 ...
journal_title:FEBS letters
pub_type: 杂志文章
doi:10.1016/0014-5793(90)80258-k
更新日期:1990-05-21 00:00:00
abstract::Using crude membrane preparations of Saccharomyces cerevisiae, we have demonstrated that glucose and glucose analogues which are not efficiently phosphorylated activate the guanine nucleotide-dependent adenylate cyclase in vitro. The activation appears to be mediated by the Ras proteins. Moreover, data are presented i...
journal_title:FEBS letters
pub_type: 杂志文章
doi:10.1016/0014-5793(91)81221-s
更新日期:1991-09-23 00:00:00
abstract::Using the polymerase chain reaction (PCR), we identified a gene family including more than 60 members which encoded similar G protein-coupled seven-transmembrane receptors. Sequence analyses of six representatives out of the 60 PCR clones showed that they had significant structural similarity to olfactory and optic re...
journal_title:FEBS letters
pub_type: 杂志文章
doi:10.1016/0014-5793(93)81302-g
更新日期:1993-02-01 00:00:00
abstract::Proteases not only play a fundamental role in numerous physiological processes, but are also involved in several human diseases including Alzheimer's disease (AD). A key protease implicated in AD is the so far unidentified gamma-secretase, which cleaves the membrane-bound beta-amyloid precursor protein (betaAPP) at th...
journal_title:FEBS letters
pub_type: 杂志文章
doi:10.1016/s0014-5793(99)01627-0
更新日期:1999-12-17 00:00:00
abstract::Two genes code for calcitonin gene-related peptides (CGRPs). One expresses by tissue-specific alternate splicing calcitonin and CGRP I mRNAs, the other CGRP II mRNA. Calcitonin is the marker of sporadic or hereditary human medullary thyroid carcinoma (MTC). CGRP II expression is not well established in normal or tumor...
journal_title:FEBS letters
pub_type: 杂志文章
doi:10.1016/0014-5793(90)80856-e
更新日期:1990-12-17 00:00:00
abstract::An experimental procedure is described for the reproducible production of hybridoma cells using the electrofusion technique. High yields can be obtained when fusion is performed in isotonic inositol solutions containing Ca2+ and Mg2+ in a ratio of 1:5 in the millimolar range. The hybridoma cells are transferred 10 min...
journal_title:FEBS letters
pub_type: 杂志文章
doi:10.1016/0014-5793(85)80315-x
更新日期:1985-03-25 00:00:00
abstract::The Golgi apparatus is known to modify and sort newly synthesized secretory proteins. However, fundamental mysteries remain about the structure, operation, and dynamics of this organelle. Important insights have emerged from studying the Golgi in yeasts. For example, yeasts have provided direct evidence for Golgi cist...
journal_title:FEBS letters
pub_type: 杂志文章,评审
doi:10.1016/j.febslet.2009.10.072
更新日期:2009-12-03 00:00:00
abstract::Endothelial cells play a pivotal role in the development of atherosclerosis. An 'activated' phenotype of these cells is manifested by signal transduction-dependent expression of genes encoding cytokines, pro- and anticoagulant factors, and cell adhesion molecules. In the current study we examined the effect of ouabain...
journal_title:FEBS letters
pub_type: 杂志文章
doi:10.1016/0014-5793(95)01301-6
更新日期:1995-12-11 00:00:00
abstract::Although transthyretin (TTR) is expressed in pancreatic alpha (glucagon) cells in the islets of Langerhans, the function of TTR in pancreatic alpha cells remains unknown. In this study, by using TTR knockout (TTR KO) mice, we determined the novel role of TTR in glucose homeostasis. We demonstrated that TTR KO mice evi...
journal_title:FEBS letters
pub_type: 杂志文章
doi:10.1016/j.febslet.2012.10.025
更新日期:2012-11-30 00:00:00
abstract::The homeodomain protein, Cdx2, has been implicated in the transcriptional regulation of genes expressed in the small intestine. In vitro studies of the carbonic anhydrase 1 (CA1) colon promoter implied that Cdx2 may also play a role in the regulation of colon-specific gene expression. The current work follows up this ...
journal_title:FEBS letters
pub_type: 杂志文章
doi:10.1016/s0014-5793(98)00103-3
更新日期:1998-02-20 00:00:00
abstract::In photosystem II (PSII) the probability that energy absorbed by core antenna chlorophyll (Chl) is transferred to the reaction center (RC) is extremely high. Although close proximity between antenna Chl ensures a high transfer efficiency, relative pigment orientation can fractionally modify it. This level of refinemen...
journal_title:FEBS letters
pub_type: 杂志文章
doi:10.1016/S0014-5793(04)00133-4
更新日期:2004-03-12 00:00:00
abstract::Ischemia/reperfusion induces nuclear factor kappaB (NF-kappaB) and AP-1 in rat hearts after 15 min of ischemia followed by reperfusion (R) for various periods of time (15 and 30 min, 1, 2, 3, 6, 12, and 24 h). Low levels of NF-kappaB and no signal for AP-1 were detected in shams and in non-ischemic tissue distant from...
journal_title:FEBS letters
pub_type: 杂志文章
doi:10.1016/s0014-5793(96)01426-3
更新日期:1997-01-13 00:00:00
abstract::When rat pancreatic islets were incubated with 10(-8) M arginine vasopressin in the presence of 15 mM glucose there was a pronounced inhibition of insulin release in comparison with controls. This inhibitory effect appeared to be specific for vasopressin since it was antagonised by vasopressin antibody. Moreover, pert...
journal_title:FEBS letters
pub_type: 杂志文章
doi:10.1016/0014-5793(88)80720-8
更新日期:1988-04-11 00:00:00
abstract::We investigated the effect of hydroxyl substituted chalcone (1a) and some chalcone analogues (1b-d) on isolated rat liver mitochondria to gain new insights into the cytotoxic mechanism of these compounds. We observed an inhibitory effect on phosphorylation and the partial uncoupling of compounds 1a and 1d. Increased r...
journal_title:FEBS letters
pub_type: 杂志文章
doi:10.1016/j.febslet.2009.11.098
更新日期:2010-02-05 00:00:00
abstract::Phospholipase C (PLC) was purified from squid retina. Soluble Gq alpha, membrane Gq alpha and G beta gamma were isolated from GTP gamma S-treated and light-illuminated photoreceptor membranes. The membrane Gq alpha stimulated phosphatidyl inositol-phospholipase C (PI-PLC) activity in a dose-dependent manner. Soluble G...
journal_title:FEBS letters
pub_type: 杂志文章
doi:10.1016/0014-5793(95)01364-4
更新日期:1995-12-27 00:00:00
abstract::The delta psi of R. sphaeroides, grown under high light to reduce the levels of light-harvesting bacteriochlorophyll, was naturally manipulated using light intensity. The relationship between delta psi and the swimming speed of free swimming populations of cells was investigated. After de-energisation by incubation in...
journal_title:FEBS letters
pub_type: 杂志文章
doi:10.1016/0014-5793(94)00572-9
更新日期:1994-07-04 00:00:00
abstract::Phosphotyrosine-containing proteins in Dictyostelium discoideum were detected by immunoblot analysis and immunoprecipitation using a monoclonal anti-phosphotyrosine antibody. The iodinated antibody recognized on bots a cluster of 205-220 kDa polypeptides and bands of 107 and 60 kDa. The 107 and 60 kDa polypeptides and...
journal_title:FEBS letters
pub_type: 杂志文章
doi:10.1016/0014-5793(90)81007-b
更新日期:1990-07-30 00:00:00
abstract::Analogues of S-prenylated cysteine like N-acetyl-S-trans,trans-farnesyl-L-cysteine (AFC) have previously been shown to inhibit the carboxyl methylation of proteins carrying a C-terminal S-prenylated cysteine residue and to block the endotoxin-activated serum-elicited chemotactic response of mouse macrophages. Here, we...
journal_title:FEBS letters
pub_type: 杂志文章
doi:10.1016/0014-5793(93)80047-x
更新日期:1993-03-15 00:00:00
abstract::There are two secondary structure models for the eukaryotic selenocysteine (Sec) tRNA(Sec). One model, the 9/4 structure, was experimentally tested and possesses acceptor and T-stems with 9 and 4 bp, respectively [Sturchler et al., 1993; Hubert et al., 1998]. The other one, the 7/5 secondary structure with a bulge in ...
journal_title:FEBS letters
pub_type: 杂志文章
doi:10.1016/s0014-5793(00)01104-2
更新日期:2000-01-28 00:00:00
abstract::The off rate from the first calcium-binding site of the C2A domain of synaptotagmin I, a putative calcium receptor in neurotransmitter release, has been determined by 15N-nuclear magnetic resonance relaxation dispersion measurements. The exchange rate was obtained by fitting the dependence of the transverse relaxation...
journal_title:FEBS letters
pub_type: 杂志文章
doi:10.1016/s0014-5793(02)02508-5
更新日期:2002-04-10 00:00:00
abstract::We amplified, using the polymerase chain reaction and calcitonin receptor (CTR) specific primers, RNA extracted from medullary thyroid carcinoma (MTC) and the derived TT cell line. Both secrete large amounts of calcitonin. Electrophoresis of amplification products revealed, in both cases, an ethidium bromide-stained b...
journal_title:FEBS letters
pub_type: 杂志文章
doi:10.1016/0014-5793(94)80503-2
更新日期:1994-04-04 00:00:00
abstract::According to the leading theory, the agent responsible for prion diseases would be the conformational isomer PrP(Sc) of a cellular protein PrP(C), the pathogenic form PrP(Sc) multiplying by converting the normal protein into a likeness of itself. The pathogenic isoform could catalyze the conformational transition so t...
journal_title:FEBS letters
pub_type: 杂志文章
doi:10.1016/s0014-5793(97)00310-4
更新日期:1997-04-21 00:00:00
abstract::Phospholipase A2 has been purified from the venom of Horned viper (Cerastes cerastes) by gel permeation chromatography followed by reverse-phase HPLC. The primary structure was established by sequence analysis of the intact protein and its enzymic peptides. The structure has 120 residues, properties like other group I...
journal_title:FEBS letters
pub_type: 杂志文章
doi:10.1016/0014-5793(91)80072-b
更新日期:1991-01-14 00:00:00
abstract::Boar semen contains a seminal plasma motility inhibitor (SPMI) that blocks the motility of demembranated-reactivated spermatozoa as well as of intact spermatozoa. In this paper, we describe the primary structure of SPMI, the coding of boar SPMI cDNA gene and its expression in various porcine tissues. Nucleotide sequen...
journal_title:FEBS letters
pub_type: 杂志文章
doi:10.1016/0014-5793(95)00701-a
更新日期:1995-07-24 00:00:00
abstract::The complete primary structure (967 amino acids) of an intestinal human aminopeptidase N (EC 3.4.11.2) was deduced from the sequence of a cDNA clone. Aminopeptidase N is anchored to the microvillar membrane via an uncleaved signal for membrane insertion. A domain constituting amino acid 250-555 positioned within the c...
journal_title:FEBS letters
pub_type: 杂志文章
doi:10.1016/0014-5793(88)80502-7
更新日期:1988-10-10 00:00:00
abstract::Ycf1p function is regulated by casein kinase 2α, Cka1p, via phosphorylation of Ser251. Cka1p-mediated phosphorylation of Ycf1p is attenuated in response to high salt stress. Previous results from our lab suggest a role for Ycf1p in cellular resistance to salt stress. Here, we show that Ycf1p plays an important role in...
journal_title:FEBS letters
pub_type: 杂志文章
doi:10.1016/j.febslet.2012.02.010
更新日期:2012-03-23 00:00:00
abstract::The functional characteristics of rat organic anion transporter OAT1 were investigated using Xenopus laevis oocytes. Uptake of p-aminohippurate (PAH) by the oocytes expressing OAT1 was markedly inhibited by glutarate, alpha-ketoglutarate and probenecid, moderately inhibited by folate and methotrexate, but not inhibite...
journal_title:FEBS letters
pub_type: 杂志文章
doi:10.1016/s0014-5793(98)01328-3
更新日期:1998-11-06 00:00:00