This selectivity was more robust when larger numbers of surroundi

This selectivity was more robust when larger numbers of surrounding items and multiple features were included in the display, and it was absent when only a few items were presented immediately outside the CRF. In addition, the popout modulation

of V4 activity was eliminated when top-down attention was directed to locations outside of the CRFs during saccade preparation, indicating that the salience of popout stimuli is not sufficient find more to drive selection by V4 neurons. These results demonstrate that neurons in feature-selective cortex are influenced by bottom-up attention, but that this influence is limited by top-down attention.”
“Whether uni- or bilateral drainage should be performed for malignant hilar biliary obstruction remains a matter of debate. Moreover, endoscopic placement of bilateral metallic stents has been considered difficult and complicated. Although the Y-stent with a central wide-open mesh facilitates bilateral stent placement, it has limitations. This study evaluated the feasibility and efficacy of the Niti-S large see more cell D-type biliary stent (LCD) with a uniform large cell for both uni- and bilateral drainage of malignant hilar biliary obstruction.\n\nFrom April

2008 to March 2009, a total of 12 consecutive patients with unresectable malignant hilar biliary obstruction of Bismuth type 2 or greater underwent placement of LCD. Before LCD placement,

all the patients underwent endoscopic unilateral biliary drainage using a plastic stent or a nasobiliary drainage tube. If jaundice improved after the procedure, the plastic stent or nasobiliary drainage tube was replaced with the unilateral LCD. If jaundice did not resolve or contralateral cholangitis occurred, bilateral LCD placement was performed.\n\nSeven patients had unilateral and five patients had bilateral LCD placement. Technical VX-680 manufacturer success was achieved for all 12 patients. An early complication occurred for one patient (8%), and stent occlusion occurred for six patients (50%) because of tumor ingrowth (n = 4) or sludge (n = 2). These patients were managed by insertion of plastic stents (n = 4) or percutaneous transhepatic biliary drainage (n = 2). The median stent patency period was 202 days.\n\nThe newly designed endoscopic metallic stent may be feasible and effective for malignant hilar biliary obstruction, and endoscopic reintervention is relatively simple.”
“Objective: A major challenge in the management of patients with complex regional pain syndrome 1 is identifying those individuals who are at risk of developing severe problems. Data from large follow-up studies providing empirical evidence are largely lacking. The goal of this study was to obtain an expert-agreed priority list of parameters that are correlated with a poor prognosis.

Results: PTHrP was expressed in cancer cells producing PTH/PTHrP

Results: PTHrP was expressed in cancer cells producing PTH/PTHrP receptor and VEGF that had invaded the bone marrow, and PTHrP was up-regulated VEGF in MDA-MB-231 in vitro. The culture medium conditioned by PTHrP-treated MDA-MB-231 cells stimulated angiogenesi.s- and osteoclastogenesis compared with control medium, giving a response

that was inhibited by VEGF-neutralizing antibody treatment. Inhibition of protein kinase C (PKC) prevented PTHrP-induced extracelhdar signal-regulated kinase (ERK1/2) and p38 activation, and PTHrP-induced VEGF expression. selleck inhibitor Conclusion: PTHrP plays an important role in modulating the angiogenic and bone osteolytic actions of VEGF through PKC-dependent activation of an ERK1/2 and p38 signaling pathway during bone metastasis by breast cancer cells.”
“We investigated the presence of beta-lactamase genes (bla) in 26 strains of Enterobacteriaceae already Dibutyryl-cAMP found positive for the qnrS1 gene, a plasmid-mediated quinolone resistance determinant. Three strains of K. pneumoniae, isolated in the period 2008-2009 at the University Hospital

in Verona, were positive for LAP-2, a narrow-spectrum beta-lactamase. These strains, namely VRB586, VRE185 and VRE196, were cultured from urine, bile and peritoneal drainage, respectively, of different patients from different units. The bla(LAP-2) and qnrS1 resistance determinant genes were separated by ISEcl2 and were located on a 97 kb conjugable and untypable plasmid, which could be transferred to a recipient strain, E. coli J53. The fluoroquinolone and ceftazidime MICs increased 1-2-fold in the transconjugant cells. The three K pneumoniae Ruboxistaurin manufacturer strains were found to be clonal by PFGE and were identified as belonging to ST147, an internationally successful clone, by MLST. The plasmid sequence, including ISEcl2 and qnrS1 genes, of K. pneumoniae ST147 was found to be highly similar to previously detected qnrS1-harbouring plasmids, suggesting the plasmid has a stable genetic

structure and that these resistance determinants have a common source. To the best of our knowledge, this is the first report of the internationally successful K pneumoniae ST147 strain carrying bla(LAP-2) and qnrS1 genes and is the first case of LAP beta-lactamase in Italy.”
“Background/aims: Liver fibrosis has been reported to be inhibited in vivo by oleanolic and ursolic acids. However, the mechanisms of the action of those triterpenoids are poorly understood. In this study, we aimed to determine the antifibrotic potential of other triterpenes, betulin and betulinic acid, and to characterize their influence on the signal transduction pathways involved in ethanol-activated hepatic stellate cells (HSCs).

We studied the right ventricular apical trabeculations

in

We studied the right ventricular apical trabeculations

in 100 adult human cadavers. Overall, we identified the moderator band in 92% of hearts. In just over two-fifths (42%), the band was a short and thick trabeculation, whereas, in one-eighth (12%), it was long and thick. In just under one-quarter of the hearts (24%), the band was short and thin, whereas it was long and thin in 14% of the hearts. In the remaining eight hearts, we were unable to identify the moderator GW786034 supplier band. The mean thickness of the band was 4.5 +/- 1.8 mm, and its mean length was 16.23 +/- 2.3 mm, ranging from 11.3 to 24.3 mm. According to these measurements, we were able to classify the band as originating less than 45% of the distance from the tricuspid valve to the apex (closer to tricuspid valve), seen in LY3023414 molecular weight 12 hearts, between 45 and 55% of the distance from the valve

to the apex, seen in 45 hearts, and greater than 550/s of this distance (closer to the apex), seen in 39 specimens. We present these data that may prove useful in the setting of the surgical repair of apical ventricular septal defects through the right atrium. Clin. Anat. 23:443-450, 2010. (C) 2010 Wiley-Liss, Inc.”
“It was studied performance, behavior and forage ingestion by beef heifers on pearl millet (Pennisetum americanum (L.) Leeke) and Alexander grass (Urochloa plantaginea) pastures from January to April 2008. The continuous grazing method with a variable number of animals was used. Forage ingestion was estimated during vegetative and reproductive stage of forage plants using chromic oxide as fecal production marker. Evaluations of behavior

were carried out by visual observation in four 24-hour continuous periods. Mean values of forage mass, forage offer and leaf blade offer were 3,927 kg/ha of DM, 14.6 kg of DM/100 kg BW and 3.36 kg of DM/100 kg BW, respectively. The variables of the pasture, animal performance, ingestive behavior and forage intake were similar for pearl millet and alexander grass. Variables of forage, animal performance NU7026 and grazing times, idle and rumination and number of bites per day showed variation over days of pasture utilization. Forage ingestion was 2.49% of the body weight and it did not vary according to phenological stage. Use of sites infested with alexander grass as pastures provides performance similar to the one obtained with pearl millet.”
“Chemotherapy-induced mucositis represents a significant burden to quality of life and healthcare costs, and may be improved through enhanced nutritional status. We first determined the safety of two nutritional drinks (plus placebo), and then potential gut protection in tumor-bearing rats in a model of methotrexate-induced mucositis. In study 1, animals were fed one of two test diets (or placebo or control chow pellets) for a total of 60 days and were monitored daily. All diets were found to be safe to administer.

With one exception, only isolates that exhibited AI-2 activity or

With one exception, only isolates that exhibited AI-2 activity or produced DPD inhibited growth of test strains. These findings demonstrate for the first time that AHLs

are present in an active coral Angiogenesis inhibitor disease. It is possible that AI-2 production among BBD and coral SML bacteria may structure the microbial communities of both a polymicrobial infection and the healthy coral microbiome.”
“With a yearly global production of about 75 million tonnes of dry matter (DM), maize stover remains a major untapped agricultural resource. While its use as a feedstock for biogas production has been well studied, the methane potential of separate single stover fraction begs further analysis. This study evaluated the composition of maize cobs, husks, leaves, and stalks and the potential of those components to produce biogas and methane. These analyses allowed an estimate of the preferred maize stover harvest chain conditions for quantity and quality. Methane yields from the fractions ranged between 206.6 and 307.0 l(N) kg(-1) VS, with husks being the most productive. In total, our estimates suggest that these fractions produce

about 3000 m(3) ha(-1) of methane from the biogas conveniently collected by different harvest chains. (C) 2014 IAgrE. Published by Elsevier Ltd. All rights reserved.”
“Biomineralisation is a well-regulated process mediated by extracellular matrix proteins. Biomimetic HDAC inhibitor remineralisation strategies should reproduce the dimension and structural hierarchy of apatite deposits within a demineralised collagen matrix. Interfibrillar and intrafibrillar remineralisation of phosphoric acid-etched human dentine was demonstrated in this study using a Portland cement/phosphate-containing fluid system in the presence of polyacrylic acid and polyvinylphosphonic acid as respective calcium phosphate- and

collagen-binding matrix protein analogues. Metastable amorphous calcium phosphate Selleckchem PF 00299804 nanoprecursors were generated when polyacrylic acid was included in the phosphate-containing fluid. When both polyvinylphosphonic acid and polyacrylic acid were included, these nanoprecursors were attracted to the acid-demineralised collagen matrix and transformed into polyelectrolyte-stabilised apatite nanocrystals that assembled along the microfibrils (intrafibrillar remineralisation) and surface of the collagen fibrils (interfibrillar remincralisation). Transition from nanocrystals to larger apatite platelets probably occurred via the formation of mesocrystal intermediates. Guided tissue remineralisation is potentially useful in the remineralisation of acid-etched dentine that is incompletely infiltrated by dentine adhesives, as well as partially demineralised caries-affected dentine. (c) 2007 Elsevier Ltd. All rights reserved.”
“Plant cell walls are complex configurations of polysaccharides that are recalcitrant to degradation.


“During critical periods of development early in life, exc


“During critical periods of development early in life, excessive or scarce nutritional environments can disrupt the development of central feeding and metabolic neural AL3818 manufacturer circuitry, leading to obesity and metabolic disorders in adulthood. A better understanding of the genetic networks that control the development of feeding and

metabolic neural circuits, along with knowledge of how and where dietary signals disrupt this process, can serve as the basis for future therapies aimed at reversing the public health crisis that is now building as a result of the global obesity epidemic. This review of animal and human studies highlights recent insights into the molecular mechanisms that regulate the development of central feeding circuitries, the mechanisms by which gestational and early postnatal nutritional status affects this process, and approaches aimed at counteracting the deleterious effects of early over-and underfeeding.”
“Synaptic plasticity deficits are increasingly recognized as causing the memory impairments which define Alzheimer’s disease (AD). In AD mouse models, evidence of abnormal synaptic function is present before the onset of cognitive deficits, and presents as increased synaptic depression revealed only when synaptic homeostasis is challenged, such

CCI-779 in vitro as with suppression of ryanodine receptor (RyR)-evoked calcium signaling. Otherwise, at early disease stages, the synaptic NVP-AUY922 molecular weight physiology phenotype appears normal. This suggests compensatory mechanisms are recruited to maintain a functionally normal net output of the hippocampal circuit.

A candidate calcium-regulated synaptic modulator is nitric oxide (NO), which acts presynaptically to boost vesicle release and glutamatergic transmission. Here we tested whether there is a feedforward cycle between the increased RyR calcium release seen in presymptomatic AD mice and aberrant NO signaling which augments synaptic plasticity. Using a combination of electrophysiological approaches, two-photon calcium imaging, and protein biochemistry in hippocampal tissue from presymptomatic 3xTg-AD and NonTg mice, we show that blocking NO synthesis results in markedly augmented synaptic depression mediated through presynaptic mechanisms in 3xTg-AD mice. Additionally, blocking NO reduces the augmented synaptically evoked dendritic calcium release mediated by enhanced RyR calcium release. This is accompanied by increased nNOS levels in the AD mice and is reversed upon normalization of RyR-evoked calcium release with chronic dantrolene treatment. Thus, recruitment of NO is serving a compensatory role to boost synaptic transmission and plasticity during early AD stages. However, NO’s dual role in neuroprotection and neurodegeneration may convert to maladaptive functions as the disease progresses.

(c) 2010 Elsevier B V All rights reserved “
“Proton beam wr

(c) 2010 Elsevier B.V. All rights reserved.”
“Proton beam writing is a direct-write technique and a promising Fedratinib datasheet method for the micromachining of commodity plastics such as acrylic resins. Herein, we describe the fabrication of microscopic devices made from a relatively thick (similar to 75 mu m) acrylic sheet using proton beam writing. In addition, a software package that converts image pixels into coordinates data was developed, and the successful fabrication of a very fine jigsaw puzzle was achieved. The size of the jigsaw puzzle pieces was 50 x 50 mu m. For practical use, a prototype of a line and space test-chart was also successfully fabricated for the

determination of spatial resolution in neutron radiography. (C) 2012 Elsevier B.V.

All rights reserved.”
“Sulfate esters and analogs are important species in synthetic chemistry, in processes catalyzed by sulfatases and in atmospheric chemistry because of their role as precursors in the formation of aerosols. In order to understand the intrinsic reactivity of these systems toward simple nucleophiles and the lack of reactivity in the sulfur center, calculations were carried out for (MeO)(2)SO2, MeS(OMe)O-2 and (MeO)(2)SO reacting with OH-, NH2-, SH-, F-, MeO- and EtO- at the B3LYP/6-311+G(3df,2p) level of theory. Our results are in agreement with previous experimental gas-phase studies that showed substitution at the C atom (S(N)[email protected]) and elimination promoted by abstraction of a proton Etomoxir purchase followed by release of formaldehyde (E(CO)2) to be the main reaction channels for dimethyl sulfate and sulfite, and proton transfer (PT) for methyl methanesulfonate. Our calculations show that the barriers for substitution at the S atom (S(N)[email protected]) are comparable,

and sometimes even lower in energy, than the barriers calculated for the S(N)[email protected] pathway for strong nucleophiles such as OH- and NH2-. Yet, S(N)[email protected] reactions are only observed in a few cases in dimethyl sulfite in reactions promoted by delocalized carbanions. Calculations that map out the energy profile of the nucleophile approaching ABT-737 solubility dmso the C and S center reveal a much more favorable energy pathway for reaction at the C center due to the electrostatic repulsion of the oxygens surrounding the S center. Therefore, reactivity at the S center in these systems is dictated-by a delicate balance between the reaction dynamics and the thermochemistry of these systems and attack of strong nucleophiles at the S center, although thermochemically favorable, is dynamically hindered and thus promote crossing over to the C route pathway. (C) 2013 Elsevier B.V. All rights reserved.”
“Examining the actual state of canopy gaps in forests is critically important for evaluating forest community structure and dynamics, and for comparing gap disturbance regimes in different forests.

In this paper, we show that TPICA is not as robust as its authors

In this paper, we show that TPICA is not as robust as its authors claim. Specifically, we discuss why TPICA’s overall objective is questionable, and we present some flaws related to the iterative nature of the TPICA algorithm. To demonstrate the relevance of these issues, we present a simulation study that compares Apoptosis inhibitor TPICA versus Parallel Factor Analysis (Parafac) for analyzing simulated multi-subject fMRI data. Our simulation

results demonstrate that TPICA produces a systematic bias that increases with the spatial correlation between the true components, and that the quality of the TPICA solution depends on the chosen ICA algorithm and iteration scheme. Thus, TPICA is not robust to small-to-moderate deviations from the model’s spatial independence assumption. In contrast, Parafac produces unbiased estimates regardless of the

spatial correlation between the true components, and Parafac with orthogonality-constrained voxel maps produces smaller biases than TPICA when the true voxel maps are moderately correlated. drug discovery As a result, Parafac should be preferred for the analysis multi-subject fMRI data where the underlying components may have spatially overlapping voxel activation patterns. (C) 2012 Elsevier B.V. All rights reserved.”
“Pituitary adenylate cyclase activating polypeptide (PACAP) and its high affinity receptor PAC1 are expressed in mammalian retina and involved in processing light information. However, their roles during retinogenesis

remain largely elusive. Previously, we have generated transgenic mice overexpressing the human PAC1 receptor, and shown that PACAP signaling is essential for normal development of the central nervous system. In this study, we show for the first time that PACAP signaling plays an important role in the development of retina, particularly in the genesis of GABAergic amacrine cells. Overexpression PF-04929113 of the PAC1 receptor leads to an early exit from retinal proliferation, reduced production of GABAergic neurons, and a marked decline in visual function. These data demonstrate that an appropriate level of PACAP signaling is required for normal retinogenesis and visual function. This finding may have implications in GABAergic neuron-related neurological conditions. (C) 2009 Elsevier Ltd. All rights reserved.”
“Background: Improving the systemic and mucosal immune response following intranasal vaccination could enhance disease protection against respiratory pathogens. We assessed the safety and immunogenicity of a novel nanoemulsion mucosal adjuvant W(80)5EC combined with approved seasonal influenza antigens.\n\nMethods: This was a first-in-human Phase I study in 199 healthy adult volunteers randomized to receive a single intranasal administration of 5%, 10%, 15% or 20% W(80)5EC, combined with 4 or 10 mu g strain-specific Fluzone (R) HA, compared with intranasal PBS, intranasal Fluzone (R), or 15 ug strain-specific intramuscular Fluzone (R).

The disparities seen in both phenotypes and selection between the

The disparities seen in both phenotypes and selection between the treatments related to the important differences in elongation timing depending on the presence of heterospecifics, although environmental covariances between traits and fitness could also contribute.\n\nPhenotypes produced by I. capensis depend on their competitive environment, and differing selection on shade-avoidance traits between competitive environments could indirectly select for increased plasticity

given gene flow between populations in different competitive contexts.\n\nNew Phytologist (2009) 183: 880-891doi: 10.1111/j.1469-8137.2009.02934.x.”
“Background Tractor overturns kill an average of 100 farmers and farm workers per year

Roll-over protective structure, (ROPS) are a proven intervention, but are not on a sufficient number of tractors in the US to reduce these deaths. Little has beet? eFT-508 price reported on ROPS use by racial minority farm operators.\n\nMethods Data from the NIOSH OISPA survey were used to assess ROPS prevalence rates from a random sample of racial minority farm operators for the year 2003, and ROPS prevalence rates from a random sample of all US farms for the year 2004.\n\nResults ROPS prevalence rates on minority farming operations follow similar patterns to ROPS prevalence rates on all US farms. A low prevalence of ROPS on farms was associated with operators over the age of 65 years, farms with small acreages, and farms operated on a part-time basis. The race of the operator had little impact on ROPS prevalence rates.\n\nConclusions Factors such GKT137831 as acreage, farm operator age, region of the US, and full- or part-time farming status influence ROPS prevalence rates Duvelisib datasheet on farms more than the race of the operator Understanding how ROPS prevalence differs across these farm and farm operator characteristics has the potential to efficiently target areas for ROPS promotion programs across the US. Am. J. Ind.

Med. 52:408-418, 2009. (C) 2009 Wiley-Liss, Inc.”
“A series of 5-substituted 2-amino-4,6-dihydroxypyrimidines were prepared by a modified condensation of the corresponding monosubstituted malonic acid diesters with guanidine in an excess of sodium ethoxide. The optimized procedure using Vilsmeier-Haack-Arnold reagent, followed by immediate deprotection of the (dimethylamino)methylene protecting groups, has been developed to convert the 2-amino-4,6-dihydroxypyrimidine analogs to novel 5-substituted 2-amino-4,6-dichloropyrimidines in high yields. Pilot screening for biological properties of the prepared compounds was done in mouse peritoneal cells using the in vitro nitric oxide (NO) assay. Irrespective of the substituent at the 5 position, 2-amino-4,6-dichloropyrimidines inhibited immune-activated NO production.

05) Floating ability for 12 h of the optimized FAMC9 was visuall

05). Floating ability for 12 h of the optimized FAMC9 was visually examined during the in vitro release studies that showed maximal drug release with zero-order

kinetics (r(2)=0.9991). ATR inhibition Thus, a novel osmotically regulated floating capsular system was developed for site-specific delivery of RHCl.”
“OBJECTIVE: To examine the use of inpatient hysterectomy and explore changes in the use of various routes of hysterectomy and patterns of referral.\n\nMETHODS: The Nationwide Inpatient Sample was used to identify all women aged 18 years or older who underwent inpatient hysterectomy between 1998 and 2010. Weighted estimates of national trends were calculated and the number of procedures performed estimated. Trends in hospital volume and across hospital characteristics were examined.\n\nRESULTS: After weighting, we identified a total 7,438,452 women who underwent inpatient hysterectomy between 1998 and 2010. The number of hysterectomies performed annually rose from 543,812 in 1998 to a peak of 681,234 in 2002; it

then declined Bromosporine consistently annually and reached 433,621 cases in 2010. Overall, 247,973 (36.4%) fewer hysterectomies were performed in 2010 compared with 2002. From 2002 to 2010 the number of hysterectomies performed for each of the following indications declined: leiomyoma (247.6%), abnormal bleeding (228.9%), benign ovarian mass (263.1%), endometriosis (265.3%), and pelvic organ prolapse (239.4%). The median hospital case volume decreased from 83 procedures per year in 2002 C59 order to 50 cases per year in 2010 (P<.001).\n\nCONCLUSION: The number of inpatient hysterectomies performed in the United States has declined substantially over the past decade. The median number of hysterectomies per hospital has declined likewise by more than 40%.”
“The bacterium Candidatus Liberibacter asiaticus (CLas) is associated with huanglongbing (HLB) in citrus in many countries. Despite the fact that many characteristics of the disease are known, the rate of multiplication of the bacterium within an infected tree is still poorly understood. To study this feature, we used the quantitative Real-Time polymerase chain reaction (Q-PCR) assay to follow and to quantify the multiplication

of CLas in grafted infected young sweet orange plants. The rate of infection by grafting reached 100% at 120 days post-inoculation (dpi) showing that grafting could easily transmit CLas. A well-adjusted linear regression equation describing the bacterial growth in planta was obtained independently with measurements taken using repeated sampling in the same plant or different plants through the analysed period. The bacterial population, measured as copy number (CN) of the 16S rDNA target gene g(-1) of tissue, increased 10,000 times from 10(3) at 30 dpi to approximately 10(8) CN at 240 dpi indicating that CLas multiplication was fastest in young citrus plants. We observed a direct relationship between the concentration of pathogen and the expression of symptoms.

From an analysis of genomic sequences for bornavirus strains isol

From an analysis of genomic sequences for bornavirus strains isolated at different time points, the time of the MRCA for bornavirus N was estimated to be smaller than 0.3 MYA. These results suggest that the integration time of itEBLN was much later than the loss of LINE-1 activity, supporting the non-LINE-1-mediated Belnacasan integration of itEBLN.”
“Carbon tetrachloride (1 ml/kg body weight as a 1:1 mixture of CCl(4) and mineral oil) was orally administered to rats. After 12 h, the activity of plasma ALT (alanine aminotransferase) was significantly higher than that of the control group, and plasma ALT and AST (aspartate aminotransferase) activities significantly

increased 24 h after CCl(4) administration. These results indicated that the necrotic process had initiated at about 12 h and developed thereafter. After 6-24 h of CCl(4) administration, the hepatic level of vitamin C, the most sensitive indicator of oxidative stress, decreased significantly, indicating that oxidative stress was significantly enhanced 6 h after CCl(4) MK-0518 in vitro intoxication and thereafter. Oral administration of vitamin E (1 ml/kg

body weight as a 1:1 mixture of alpha-tocopherol and mineral oil) 12 h before CCl(4) administration caused a significant elevation of liver vitamin E level and ameliorated liver necrosis 24 h after CCl(4) intoxication based on plasma AST and ALT. Vitamin E also significantly restored the hepatic vitamin C concentration 12 and 24 h after CCl(4) intoxication, demonstrating that vitamin E functioned as an antioxidant. The liver vitamin E concentration was not changed by vitamin E supplementation to rats that did not receive CCl(4). This result indicated that vitamin E accumulated in the damaged liver. The activation of JNK, ERK1/2 and p38 MAPK took place 1.5 h after CCl(4) administration. Co-administration of alpha-tocopherol with CCl(4) did not

affect these early changes in MAPKs.”
“Friedreich’s ataxia (FRDA) is an autosomal recessive disease caused by mutations that produce a deficiency in frataxin. Despite the importance of neurodegeneration in FRDA, little is known about the consequences buy Etomoxir of frataxin deficiency in neuronal cells. Here we describe a neuronal cell model for FRDA based on the use of lentiviral vectors that carry minigenes encoding frataxin-specific shRNAs that silence the expression of this gene. These lentivectors can knockdown frataxin expression in human neuroblastoma SH-SY5Y cells, which results in large-scale cell death in differentiated neuron-like cells but not in undifferentiated neuroblastoma cells. Frataxin-deficient neuron-like cells appear to die through apoptosis that is accompanied by up-regulation of p53, PUMA and Bax and activation of caspase-3.