Miracle-dz/UniFAR-FTUs huggingface dataset:
Charcot‐Marie‐Tooth 1A: A narrative review with clinical and anatomical perspectives

Charcot–Marie‐Tooth 1A (CMT1A) is regarded as the most common hereditary peripheral neurodegenerative disorder. This narrative review highlights perspectives around the historically well‐established and characteristic anatomical manifestations of CMT1A seen in the feet, legs and hands, in addition to a clinical diagnosis that may be confirmed by electrophysiology, genetic or molecular markers together with the presence of a typical family history. A less well‐known perspective is the potential for systemic manifestations and wider complication. The condition is characterised by a progressive clinical picture with unmistakable anatomical and neurological features that have been described since the late 19th century. There remains no cure although supportive, rehabilitative, and surgical regimes may provide helpful management or amelioration of symptoms. Most recently, the emergence of a pleotherapeutic approach suggests distinct promise. Future research focused on a detailed elucidation of the underlying molecular mechanisms underpinning myelin and axonal function may eventually hold the key to successful treatment of CMT1A. Genetic modification would potentially present a cure. Clin. Anat. 29:547–554, 2016. © 2015 Wiley Periodicals, Inc.
Capacitive Circuit Method for Fast and Efficient Design of Wideband Radar Absorbers

A simple, fast and efficient method for designing wideband radar absorbers is proposed. The idea is to modify the circuit analog absorber method without perturbing the bandwidth. This is done by utilizing the asymptotic behavior of such an absorber at low frequency and replacing the band-stop resonating frequency selective surfaces with low-pass capacitive ones, which can be synthesized by square patches. It is shown that higher frequencies are not influenced by these modifications. A thin wideband capacitive circuit absorber (CCA) is presented with 28% reduction of thickness and 57% increase of bandwidth in comparison to the Salisbury screen. It is also explained why some optimized metamaterial designs fail to compete with the CCA method. For high permittivity layers, it is shown that the CCA is a better solution than the Jaumann absorber and improvements both in thickness and bandwidth are possible. A three layered ultrawideband CCA is presented with total thickness of 15.1 mm.
VARIATION IN THE PRE-TRANSIT BALMER LINE SIGNAL AROUND THE HOT JUPITER HD 189733B

As followup to our recent detection of a pre-transit signal around HD 189733 b, we obtained full pre-transit phase coverage of a single planetary transit. The pre-transit signal is again detected in the Balmer lines but with variable strength and timing, suggesting that the bow shock geometry reported in our previous work does not describe the signal from the latest transit. We also demonstrate the use of the Ca ii H and K residual core flux as a proxy for the stellar activity level throughout the transit. A moderate trend is found between the pre-transit absorption signal in the 2013 data and the Ca ii H flux. This suggests that some of the 2013 pre-transit hydrogen absorption can be attributed to varying stellar activity levels. A very weak correlation is found between the Ca ii H core flux and the Balmer line absorption in the 2015 transit, hinting at a smaller contribution from stellar activity compared to the 2013 transit. We simulate how varying stellar activity levels can produce changes in the Balmer line transmission spectra. These simulations show that the strength of the 2013 and 2015 pre-transit signals can be reproduced by stellar variability. If the pre-transit signature is attributed to circumplanetary material, its evolution in time can be described by accretion clumps spiraling toward the star, although this interpretation has serious limitations. Further high-cadence monitoring at Hα is necessary to distinguish between true absorption by transiting material and short-term variations in the stellar activity level.
Effects of GonaCon Immunocontraceptive Vaccine in Free-Ranging Female Rocky Mountain elk (Cervus elaphus nelsoni)

Duration of efficacy and prevalence of side-effects associated with GonaCon Immunocon- traceptive Vaccine (GonaCon) in free-ranging female elk (Cervus elaphus) are unknown. In January 2008, we captured120 mature femaleelk inRocky MountainNational Park(CO,USA),determined pregnancystatus, and randomly assigned them to treated (n ¼60; 1.5mL of GonaCon) or control (n ¼60; 1.5mL of saline) groups. During the following 3 winters we recaptured, collected blood for antibody concentrations, and euthanized 10-20 elk in each group. At necropsy, we determined pregnancy and collected tissues from organs associated with the hypothalamic-pituitary-gonadal axis. We relocated injection sites, collected muscle tissue, and performed bacterial culture when inflammation was present. Proportion of pregnant elk among control females ranged from 0.75 to 0.90. Proportion pregnant after treatment with GonaCon was 0.00 (95% CI ¼0.0-0.22) in year 1, 0.31 (CI ¼0.09-0.61) in year 2, and 0.65 (CI ¼0.41-0.85) in year 3. Antibody concentrations were higher in non-pregnant than pregnant treated females. We found no antemortem evidence of lameness or swelling at the injection site; however, at necropsy all treated females had pyogranulomatous inflammation at the injection site. We observed no consistent changes within the hypothalamic-pituitary-gonadal axis. We conclude that GonaCon is effective at reducing pregnancy for 1-2 years post-vaccination and is strongly associated with sterile inflammation at the site of injection. Similar to other species, the vaccine is less effective in elk under free-ranging conditions than those in a captive environment. 2014 The Wildlife Society.
New Export Pathway in Plasmodium falciparum‐Infected Erythrocytes: Role of the Parasite Group II Chaperonin, PfTRiC

The export of numerous proteins to the plasma membrane of its host erythrocyte is essential for the virulence and survival of the malaria parasite Plasmodium falciparum. The Maurer's clefts, membrane structures transposed by the parasite in the cytoplasm of its host erythrocyte, play the role of a marshal platform for such exported parasite proteins. We identify here the export pathway of three resident proteins of the Maurer's clefts membrane: the proteins are exported as soluble forms in the red cell cytoplasm to the Maurer's clefts membrane in association with the parasite group II chaperonin (PfTRIC), a chaperone complex known to bind and address a large spectrum of unfolded proteins to their final location. We have also located the domain of interaction with PfTRiC within the amino‐terminal domain of one of these Maurer's cleft proteins, PfSBP1. Because several Maurer's cleft membrane proteins with different export motifs seem to follow the same route, we propose a general role for PfTRiC in the trafficking of malarial parasite proteins to the host erythrocyte.
Seed bank dynamics of a semi-arid coastal shrub community in Pakistan

Abstract The relationship between the seed bank and existing vegetation was studied. Seed bank flora and existing vegetation show a high degree of similarity and Cressa cretica dominated both; a few rare species were also present. Seasonal variation in the seed bank was also studied for the 1989–90 and 1990–91 growing seasons. Most species demonstrated the persistent nature of the seed bank with their numbers increasing after seed dispersal and decreasing in subsequent months. The seed bank of a coastal community in Sindh, Pakistan could be used to predict existing vegetation.
Polynomial modules over the Steenrod algebra and conjugation in the Milnor basis

Let Ps = F2 [x1, . , xs] be the mod 2 cohomology of the s-fold product of RP∞ with the usual structure as a module over the Steenrod algebra. A monomial in Ps is said to be hit if it is in the image of the action A ⊗ Ps → Ps where A is the augmentation ideal of A. We extend a result of Wood to determine a new family of hit monomials in Ps. We then use similar methods to obtain a generalization of antiautomorphism formulas of Davis and Gallant.
Production of Soluble Triggering Receptor Expressed on Myeloid Cells by Lipopolysaccharide-Stimulated Human Neutrophils Involves De Novo Protein Synthesis

ABSTRACT The triggering receptor expressed on myeloid cells (TREM-1) is a recently identified receptor expressed on neutrophils and monocytes. Activation of the receptor induces neutrophils to release the enzyme myeloperoxidase and inflammatory cytokines such as interleukin-8. TREM-1 has an alternatively spliced variant that lacks the transmembrane region, resulting in the receptor being secreted in a soluble form (sTREM-1). Soluble TREM-1 has been detected in plasma during experimental and clinical sepsis and has been advocated as a diagnostic marker of infection for pneumonia and as a prognostic marker for patients with septic shock. We studied TREM-1 surface expression, using flow cytometry, and simultaneously measured sTREM-1 concentrations in culture supernatants of lipopolysaccharide (LPS)-stimulated neutrophils. TREM-1 surface expression was constitutive and was not upregulated upon LPS stimulation. However, sTREM-1 release from neutrophils was significantly upregulated by LPS stimulation (P < 0.0001), an effect that was abrogated by cycloheximide. Soluble TREM-1 is therefore secreted by human neutrophils in response to LPS challenge in a process involving de novo protein synthesis that is not accompanied by an upregulation of the TREM-1 receptor on the surfaces of the cells.
A survey of the occurrence of agaritine in U.K. cultivated mushrooms and processed mushroom products.

The naturally occurring compound agaritine (beta-N-[gamma-L(+)glutamyl]-4-hydroxy-methylphenyl-hydrazine) has been determined in fresh, dried and processed mushrooms. A method was developed involving extraction of the toxin into methanol, clean-up where appropriate (for processed products) by high-performance size exclusion liquid chromatography and determination by reverse-phase HPLC with electrochemical detection. Diode array UV monitoring was used for confirmation. The method had a recovery of 90-98%, a relative standard deviation of 3-5% and a limit of detection of agaritine of 5 mg/kg on a dry-weight basis. Fresh cultivated mushrooms showed agaritine levels of 100-250 mg/kg and 80-190 mg/kg for two different commercial strains. There were slight differences in levels of agaritine between mushrooms of different sizes, and between those of the same size but harvested at different times (different breaks). Retail processed mushrooms products had low agaritine levels in the range 6-33 mg/kg, with the exception of one dried sliced mushroom product found to contain 6520 mg/kg.
Serum Endocan Levels are Associated With Paraoxonase 1 Concentration in Patients With Chronic Kidney Disease

Endocan is a soluble proteoglycan released by the vascular endothelium. The increase of its serum levels is associated with inflammation, endothelial dysfunction and cardiovascular events in patients with chronic kidney disease (CKD). We studied the association of serum endocan with the lipid profile of 105 CKD patients with dyslipidemia, divided in two groups, non‐dialyzed (CKD, N = 57) and hemodialysis (HD, N = 48) in comparison with 30 normal controls (NC). We also analyzed endocan in relation with the concentration of two serum HDL‐linked members of the paraoxonase (PON) family, PON1 and PON3, which have been previously found to have antiatherogenic properties. The results showed that endocan levels were significantly higher in HD patients than in CKD patients (P < 0.001) and NC (P < 0.001). PON1 was significantly decreased only in HD patients compared to NC (P < 0.001), whereas PON3 was significantly increased in both patient groups (P < 0.001). Endocan levels were significantly and positively correlated with total cholesterol and LDL‐C in CKD and additionally were negatively correlated with HDL‐C in HD group. PON1 levels were significantly correlated with endocan in both groups, while no correlation was observed for PON3 in either group. Multiple regression analysis between endocan and the above lipid parameters in the total of patients revealed that endocan was independently associated only with PON1 (β = −0.513, P = 0.002). It is concluded that the increase of serum endocan levels in patients with CKD may be associated with the decrease of PON1 concentration, irrespective of lipid alterations produced by atherosclerosis development.
Effect of Presymptomatic Body Mass Index and Consumption of Fat and Alcohol on Amyotrophic Lateral Sclerosis.

IMPORTANCE Because dietary intake may influence pathophysiologic mechanisms in sporadic amyotrophic lateral sclerosis (ALS), the association between premorbid dietary intake and the risk of sporadic ALS will provide insight into which mechanisms are possibly involved in ALS pathophogenesis. OBJECTIVE To systematically determine the association between premorbid dietary intake and the risk of sporadic ALS. DESIGN, SETTING, AND PARTICIPANTS A population-based case-control study was conducted in a general community setting in the Netherlands from January 1, 2006, to September 30, 2011. Analysis was conducted April 1, 2013, to November 15, 2014. All patients with a new diagnosis of possible, probable (laboratory supported), or definite ALS according to the revised El Escorial criteria were included and multiple sources were used to ensure complete case ascertainment. Of 986 eligible patients, 674 gave informed consent and returned a complete questionnaire; 2093 controls randomly selected from the general practitioners' registers and frequency matched to the patients for sex and age were included. MAIN OUTCOMES AND MEASURES We studied the premorbid intake of nutrients in association with the risk of ALS by using a 199-item food frequency questionnaire adjusted for confounding factors and corrected for multiple comparisons while minimizing recall bias. RESULTS Presymptomatic total daily energy intake in patients, reported as mean (SD), was significantly higher compared with controls (2258 [730] vs 2119 [619] kcal/day; P < .01), and presymptomatic body mass index (calculated as weight in kilograms divided by height in meters squared) was significantly lower in patients (25.7 [4.0] vs 26.0 [3.7]; P = .02). With values reported as odds ratio (95% CI), higher premorbid intake of total fat (1.14; 1.07-1.23; P < .001), saturated fat (1.43; 1.25-1.64; P < .001), trans-fatty acids (1.03; 1.01-1.05; P < .001), and cholesterol (1.08; 1.05-1.12; P < .001) was associated with an increased risk of ALS; higher intake of alcohol (0.91; 0.84-0.99; P = .03) was associated with a decreased risk of ALS. These associations were independent of total energy intake, age, sex, body mass index, educational level, smoking, and lifetime physical activity. No significant associations between dietary intake and survival were found. CONCLUSIONS AND RELEVANCE The combination of independent positive associations of a low premorbid body mass index and a high fat intake together with prior evidence from ALS mouse models transgenic for SOD1 and earlier reports on premorbid body mass index support a role for increased resting energy expenditure before clinical onset of ALS.
Identification of the Hard X-Ray Source Dominating the E > 25 keV Emission of the Nearby Galaxy M31

We report the identification of a bright hard X-ray source dominating the M31 bulge above 25 keV from a simultaneous NuSTAR–Swift observation. We find that this source is the counterpart to Swift J0042.6+4112, which was previously detected in the Swift BAT All-sky Hard X-ray Survey. This Swift BAT source had been suggested to be the combined emission from a number of point sources; our new observations have identified a single X-ray source from 0.5 to 50 keV as the counterpart for the first time. In the 0.5–10 keV band, the source had been classified as an X-ray Binary candidate in various Chandra and XMM-Newton studies; however, since it was not clearly associated with Swift J0042.6+4112, the previous E < 10 keV observations did not generate much attention. This source has a spectrum with a soft X-ray excess (kT ∼ 0.2 keV) plus a hard spectrum with a power law of and a cutoff around 15–20 keV, typical of the spectral characteristics of accreting pulsars. Unfortunately, any potential pulsation was undetected in the NuSTAR data, possibly due to insufficient photon statistics. The existing deep HST images exclude high-mass (>3 ) donors at the location of this source. The best interpretation for the nature of this source is an X-ray pulsar with an intermediate-mass (<3 ) companion or a symbiotic X-ray binary. We discuss other possibilities in more detail.
A Quantum von Neumann Architecture for Large-Scale Quantum Computing in Systems with Long Coherence Times, such as Trapped Ions

As the size of quantum systems becomes bigger, more complicated hardware is required to control these systems. In order to reduce the complexity, I discuss the amount of parallelism required for a fault-tolerant quantum computer and what computation speed can be achieved in different architectures. To build a large-scale quantum computer, one can use architectural principles, from classical computer architecture, like multiplexing or pipelining. In this document, a Quantum von Neumann architecture is introduced which uses specialized hardware for the different tasks of a quantum computer, like computation or storage. Furthermore, it requires long qubit coherence and the capability to move quantum information between the different parts of the quantum computer. As an example, a Quantum von Neumann architecture for trapped ions is presented which incorporates multiplexing in the memory region for large-scale quantum computation. To illustrate the capability of this architecture, a model trapped ion quantum computer based on Quantum von Neumann architecture, the Quantum 4004, is introduced. Its hardware is optimized for simplicity and uses the classical Intel 4004 CPU from 1971 as a blueprint. The Quantum 4004 has only a single processing zone and is structured in 4 qubit packages. Its quantum memory can store up to 32768 qubit ions and its computation speed is 10 $\mu$s for single qubit operations and 20 $\mu$s for two-qubit operations.
Platelet count in malaria patients.

BACKGROUND Malaria is a major health problem in the tropics with high morbidity and mortality. This study is conducted to analyse the effects of malaria on platelets. METHODS This prospective study was conducted on 200 diagnosed cases of malaria in Department of Medicine, LUMHS, Jamshoro/Hyderabad from February to December 2010. The diagnosis of malaria was carried out by thin and thick blood films. Platelet count was performed using an automated counter. Thrombocytopenia was classified as mild (50-150x10(3) cells/microl), moderate (20-50x10(3) cells/microl) and severe (<20x10(3) cell/microl). RESULTS The age of patients ranged from 16 to 80 (28 +/- 10.5) years, median age was 30 years. Among the study population, males were 124 (62%) and females were 76 (38%). Out of them 105 (52.5%) were cases of Plasmodium falciparum, 93 (46.5%) were of P. vivax, and 2 (1%) were of P. malaria. The data showed that 171 (85.5%) patients were having low platelet count; 141 (70.5%) had mild, 21 (10.5%) moderate, and 9 (4.5%) had severe thrombocytopenia. Twenty-nine (14.5%) patients had normal platelet count. CONCLUSION Malaria is associated with different degrees of low platelet count with rarely increased bleeding tendency.
Aging and computational systems biology

Aging research is undergoing a paradigm shift, which has led to new and innovative methods of exploring this complex phenomenon. The systems biology approach endeavors to understand biological systems in a holistic manner, by taking account of intrinsic interactions, while also attempting to account for the impact of external inputs, such as diet. A key technique employed in systems biology is computational modeling, which involves mathematically describing and simulating the dynamics of biological systems. Although a large number of computational models have been developed in recent years, these models have focused on various discrete components of the aging process, and to date no model has succeeded in completely representing the full scope of aging. Combining existing models or developing new models may help to address this need and in so doing could help achieve an improved understanding of the intrinsic mechanisms which underpin aging. WIREs Syst Biol Med 2016, 8:123–139. doi: 10.1002/wsbm.1328
Effects of Chemotherapy Regimen and Radiation Modality on Hematologic Toxicities in Patients Receiving Definitive Platinum-based Doublet Chemoradiation for Non–Small Cell Lung Cancer

Objectives: Predictors of acute hematologic toxicities during definitive chemoradiation for non–small cell lung cancer (NSCLC) are incompletely defined. Materials and Methods: We retrospectively analyzed 604 patients treated with definitive platinum-based doublet chemoradiation therapy for stage III NSCLC. The outcome of interest was grade ≥3 acute hematologic toxicities, specifically white blood cell, hemoglobin, platelet, neutrophil, and lymphocyte decrease during chemoradiation therapy. We assessed the association between any grade ≥3 acute hematologic toxicity with patient demographic, disease, radiation factors (specifically modality and dose), and chemotherapy agents via stepwise multivariate logistic regression. Survival was compared via log-rank and univariate Cox regression analyses. Results: There was no significant association between radiation modality and any hematologic toxicity on multivariate analysis. However, use of etoposide was found to be significantly associated with white blood cell, platelet, and neutrophil decrease compared with paclitaxel and docetaxel (all P<0.05). No differences were found between platinum agents. Overall survival (OS) and event-free survival (EFS) were significantly worse in patients who experienced grade ≥3 hemoglobin (OS: hazard ratio [HR]=1.5; 95% confidence interval [CI], 1.05-2.26; P=0.03, EFS: HR=1.7; 95% CI, 1.2-2.4; P=0.0032) and lymphocyte (OS: HR=1.5; 95% CI, 1.1-2.1; P=0.01, EFS: HR=1.4; 95% CI, 1.1-1.9; P=0.02) decreases. Conclusions: Chemotherapy identity, specifically the nonplatinum agent, was significantly associated with grade ≥3 hematologic toxicities, whereas radiation modality was not.
Genetic epidemiology of Alzheimer disease.

Dementia is a major health problem in the elderly. Following an extended period of loss of personality and cognition, the disease results in a state of complete dependency. By far the most common cause of dementia is Alzheimer disease, which is clinically characterized by a gradual, progressive decline in intellectual functions (1). Psychosis, depression, agitation, and anxiety are common manifestations (2). The most frequently used diagnostic criteria for Alzheimer disease are described by the National Institute of Neurological and Communicative Disorders and Stroke and the Alzheimer's Disease and Related Disorders Association (NINCDS-ADRDA) (3). The diagnosis of Alzheimer disease is considered to be probable when alternative causes of dementia are excluded (3, 4). According to the NINCDS-ADRDA criteria, a definite diagnosis is made when a probable diagnosis of Alzheimer disease is confirmed at autopsy (3). The neuropathologic characteristics of Alzheimer disease are senile plaques, neurofibrillary tangles, amyloid angiopathy, neuronal loss, as well as decreased activity of the enzyme choline acetyltransferase (5). Senile plaques are extracellular deposits of predominantly /3-amyloid. Neurofibrillary tangles are intraneuronal inclusions, which are, in part, composed of abnormally phosphorylated tau protein. The prevalence of Alzheimer disease increases with advancing age. It affects less than 1 percent of individuals aged 60-64 years, and up to 40 percent of those over age 85 years (6). Also, the incidence increases with aging, and is estimated to be 1 per 1,000