Miracle-dz/UniFAR-FTUs huggingface dataset:
Evaluation of orofacial motricity in patients with mucopolysaccharidosis: a cross-sectional study.

OBJECTIVES To characterize the stomatognathic system and stomatognathic functions in patients with mucopolysaccharidosis. METHODS Cross-sectional and observational study of patients with mucopolysaccharidosis seen at the outpatient clinic at the Medical Genetics Service of Hospital de Clínicas de Porto Alegre. The inclusion criteria were the existence of a biochemical or molecular diagnosis of any type of mucopolysaccharidosis and the agreement to participate in the study by signing an informed consent form. Seventy-eight patients were evaluated through phonoaudiological anamnesis and physical exam. RESULTS Alterations in at least one item of each structure of the stomatognathic system or stomatognathic function were found in all patients who allowed evaluation of both items on physical examination (n = 76/78). The most frequently compromised structures and functions were respectively the dental arch and the tongue, swallowing and mastication. The only statistically significant difference found between types of mucopolysaccharidosis involved the habitual position of the tongue between the teeth (most frequent in mucopolysaccharidosis VI). Among patients with mucopolysaccharidosis I, II or VI who underwent enzyme replacement therapy or not, there was statistically significant difference in oral breathing mode (more frequent in the group without enzyme replacement therapy). CONCLUSIONS Alterations in stomatognathic systems and functions are prevalent among individuals with mucopolysaccharidosis, even if enzyme replacement therapy is administered. Such finding suggests that speech therapy follow-up plays a major role in the treatment plan of this group of diseases; this hypothesis should be confirmed by additional studies.
On some combinatorial problems in metric spaces of bounded doubling dimension

A metric space has doubling dimension d if for every ρ > 0, every ball of radius ρ can be covered by at most 2d balls of radius ρ/2. This generalizes the Euclidean dimension, because the doubling dimension of Euclidean space Rd is proportional to d. The following results are shown, for any d ≥ 1 and any metric space of size n and doubling dimension d: First, the maximum number of diametral pairs is Θ(n2). Second, if d = 1, the maximum possible weights of the minimum spanning tree and the all-nearest neighbors graph are Θ(R log n) and Θ(R), respectively, where R is the minimum radius of any ball containing all elements of the metric space. Finally, if d > 1, the maximum possible weights of both the minimum spanning tree and the all-nearest neighbors graph are Θ(Rn1−1/d). These results show that, for 1 ≤ d ≤ 3, metric spaces of doubling dimension d behave differently than their Euclidean counterparts.
Synaptic Integration in Striate Cortical Simple Cells

Simple cells in the visual cortex respond to the precise position of oriented contours (Hubel and Wiesel, 1962). This sensitivity reflects the structure of the simple receptive field, which exhibits two sorts of antagonism between on andoff inputs. First, simple receptive fields are divided into adjacent on and off subregions; second, within each subregion, stimuli of the reverse contrast evoke responses of the opposite sign: push–pull (Hubel and Wiesel, 1962;Palmer and Davis, 1981; Jones and Palmer, 1987; Ferster, 1988). We have made whole-cell patch recordings from cat area 17 during visual stimulation to examine the generation and integration of excitation (push) and suppression (pull) in the simple receptive field. The temporal structure of the push reflected the pattern of thalamic inputs, as judged by comparing the intracellular cortical responses to extracellular recordings made in the lateral geniculate nucleus. Two mechanisms have been advanced to account for the pull–withdrawal of thalamic drive and active, intracortical inhibition (Hubel and Wiesel, 1962; Heggelund, 1986; Ferster, 1988). Our results suggest that intracortical inhibition is the dominant, and perhaps sole, mechanism of suppression. The inhibitory influences operated within a wide dynamic range. When inhibition was strong, the membrane conductance could be doubled or tripled. Furthermore, if a stimulus confined to one subregion was enlarged so that it extended into the next, the sign of response often changed from depolarizing to hyperpolarizing. In other instances, the inhibition modulated neuronal output subtly, by elevating spike threshold or altering firing rate at a given membrane voltage.
Molecular Systems Biology Peer Review Process File Coupled Pre-mrna and Mrna Dynamics Unveil Operational Strategies Underlying Transcriptional Responses to Stimuli Transaction Report

(Note: With the exception of the correction of typographical or spelling errors that could be a source of ambiguity, letters and reports are not edited. The original formatting of letters and referee reports may not be reflected in this compilation.) Thank you again for submitting your work to Molecular Systems Biology. We have now heard back from the three referees whom we asked to evaluate your manuscript. As you will see from the reports below, the referees find the topic of your study of potential interest and they appreciate your approach. However, they raise several concerns on your work, which should be convincingly addressed in a revision of the present work. The recommendations provided by the reviewers are very clear in this regard. In particular, there is a need for a more rigorous and detailed presentation of the computational analysis and to justify or even relax some of the key assumptions used in the analysis (eg as constant degradation rates or consistent elongation rates). Finally, reviewer #3 makes the relevant point that a deeper functional analysis of the results would strengthen the biological insights gained from this study. Systems Biology will publish online a Review Process File to accompany accepted manuscripts. When preparing your letter of response, please be aware that in the event of acceptance, your cover letter/point-by-point document will be included as part of this File, which will be available to the scientific community. More information about this initiative is available in our Instructions to Authors. If you have any questions about this initiative, please contact the editorial office msb@embo.org. If you feel you can satisfactorily deal with these points and those listed by the referees, you may wish to submit a revised version of your manuscript. Please attach a covering letter giving details of the way in which you have handled each of the points raised by the referees. A revised manuscript
Morphology of the mechanosensory lateral line system in the Atlantic Stingray, Dasyatissabina: The mechanotactile hypothesis

The biological function of anatomical specializations in the mechanosensory lateral line of elasmobranch fishes is essentially unknown. The gross and histological features of the lateral line in the Atlantic stingray, Dasyatis sabina, were examined with special reference to its role in the localization and capture of natural invertebrate prey. Superficial neuromasts are arranged in bilateral rows near the dorsal midline from the spiracle to the posterior body disk and in a lateral position along the entire length of the tail. All dorsal lateral line canals are pored, contain sensory neuromasts, and have accessory lateral tubules that most likely function to increase their receptive field. The pored ventral canal system consists of the lateral hyomandibular canal along the disk margin and the short, separate mandibular canal on the lower jaw. The extensive nonpored and relatively compliant ventral infraorbital, supraorbital, and medial hyomandibular canals form a continuous complex on the snout, around the mouth, and along the abdomen. Vesicles of Savi are small mechanosensory subdermal pouches that occur in bilateral rows only along the ventral midline of the rostrum. Superficial neuromasts are best positioned to detect water movements along the transverse body axis such as those produced by tidal currents, conspecifics, or predators. The pored dorsal canal system is positioned to detect water movements created by conspecifics, predators, or possibly distortions in the flow field during swimming. Based upon the stingray lateral line morphology and feeding behavior, we propose the Mechanotactile Hypothesis, which states that the ventral nonpored canals and vesicles of Savi function as specialized tactile mechanoreceptors that facilitate the detection and capture of small benthic invertebrate prey. J. Morphol. 238:1–22, 1998. © 1998 Wiley‐Liss, Inc.
Scalable facility placement for communication cost reduction in wireless networks

Wireless network nodes, recently powerful enough to assume sophisticated roles, have changed the angle of studying service communication costs in modern, typically large-scale and inherently dynamic, network environments. Communication cost minimization for using a certain service, is one of the challenging issues in these environments. Its ensuing optimization problem is not only difficult (NP-hard and requires global information), but also its centralized solution is non-scalable. The approach followed in this paper is a distributed one based on local information. First, a facility replication method ensuring overall communication cost reduction is proposed and analyzed. No additional overhead is introduced and the cost of hosting a new facility is also taken into account. Second, and aiming at reducing the communication cost for using a service, a policy that employs facility replication in conjunction with facility migration, is introduced. This policy is easy to implement since it relies on information locally available at the facility node, and its efficiency and limitations are also analyzed and discussed here. Simulation results are presented, supporting the analytical findings and demonstrating a significant overall cost reduction when the proposed policy is implemented.
Quasi-isometric rigidity of nonuniform lattices in higher rank symmetric spaces

Thus φ is bi-Lipschitz on large scales. If there exists a constant C ′ such that every point of Y is within a distance C′ of a point of φ(X), then there is a quasi-isometric embedding ψ : Y → X which is a “coarse inverse of φ”, i.e. supx∈X dX(x, ψ(φ(x))) <∞ and supy∈Y dY (y, φ(ψ(y))) < ∞. In this case φ is called a quasi-isometry and the spaces X and Y are said to be quasi-isometric. A basic example to keep in mind is that the fundamental group π1(M) (endowed with the word metric) of a compact Riemannian manifold M is quasi-isometric to the universal cover M̃ of M . In this paper we prove the following theorem:
Hypercoagulable States in Cardiovascular Disease

Certain individuals may have an abnormal propensity to develop venous or arterial thrombosis and either experience thromboembolic events relatively early in life or suffer recurring events.1 This well-described clinical phenomenon, paralleled by familial clustering of thrombotic phenotypes, has led to a comprehensive search for inherited and acquired forms of hypercoagulability as part of the condition known as thrombophilia. Although clearly defined associations have been described for hypercoagulable states and thrombosis within the venous system, the establishment of causative or contributing roles for these same thrombophilic conditions and the occurrence of arterial thrombosis has been considerably more elusive.2 The World Health Organization/International Society of Thrombosis and Hemostasis in 1995 defined thrombophilia as an unusual tendency toward thrombosis.1 Frequently cited features traditionally include (1) early age of onset; (2) recurrent episodes; (3) strong family history; (4) unusual, migratory, or widespread locations; and (5) severity out of proportion to any recognized stimulus. Here, we provide an updated review of hypercoagulable states in cardiovascular disease in 3 sections: (1) inherited hypercoagulable states; (2) acquired hypercoagulable states; and (3) diagnosis and management. Establishment of the role of pathways that lead to heritable hypercoagulable phenotypes in multifactorial disorders such as cardiovascular diseases is complicated by the inability to adequately discern the necessity and sufficiency of proposed mediators of hypercoagulability (Figure 1). 2 Hyperhomocysteinemia is a good example of the challenges faced.3 Elevated homocysteine levels exert numerous vasotoxic effects on the endothelium, which lead to endothelial cell dysfunction, platelet activation, and thrombus formation and an increased risk of thrombotic events.4 A C677T point mutation within the coding region of the methylenetetrahydrofolate reductase ( MTHFR ) gene is the most common genetic cause of hyperhomocysteinemia, with homozygotes for the 677T allele exhibiting mild to moderate elevations of serum homocysteine and a varying propensity for …
Targeting Bone Alleviates Osteoarthritis in Osteopenic Mice and Modulates Cartilage Catabolism

Objective Subchondral bone modifications occur early in the development of osteoarthritis (OA). The level of bone resorption might impact cartilage remodeling. We therefore assessed the in vivo and in vitro effects of targeting bone resorption in OA and cartilage metabolism. Methods OA was induced by meniscectomy (MNX) in ovariectomized osteopenic mice (OP) treated with estradiol (E2), pamidronate (PAM), or phosphate buffered saline (PBS) for 6 weeks. We assessed the subchondral bone and cartilage structure and the expression of cartilage matrix proteases. To assess the involvement of bone soluble factors in cartilage metabolism, supernatant of human bone explants pre-treated with E2 or PAM were transferred to cartilage explants to assess proteoglycan release and aggrecan cleavage. OPG/RANKL mRNA expression was assessed in bone explants by real-time quantitative PCR. The role of osteoprotegerin (OPG) in the bone-cartilage crosstalk was tested using an OPG neutralizing antibody. Results Bone mineral density of OP mice and osteoclast number were restored by E2 and PAM (p<0.05). In OP mice, E2 and PAM decreased ADAMTS-4 and -5 expression, while only PAM markedly reduced OA compared to PBS (2.0±0.63 vs 5.2±0.95; p<0.05). OPG/RANKL mRNA was increased in human bone explants treated with both drugs (2.2–3.7-fold). Moreover, supernatants from bone explants cultured with E2 or PAM reduced aggrecan cleavage and cartilage proteoglycan release (73±8.0% and 80±22% of control, respectively, p<0.05). This effect was reversed with osteoprotegerin blockade. Conclusion The inhibition of bone resorption by pamidronate in osteopenic mice alleviates the histological OA score with a reduction in the expression of aggrecanases. Bone soluble factors, such as osteoprotegerin, impact the cartilage response to catabolic factors. This study further highlights the importance of subchondral bone in the regulation of joint cartilage damage in OA.
Power Management and Power Flow Control With Back-to-Back Converters in a Utility Connected Microgrid

This paper proposes a method for power flow control between utility and microgrid through back-to-back converters, which facilitates desired real and reactive power flow between utility and microgrid. In the proposed control strategy, the system can run in two different modes depending on the power requirement in the microgrid. In mode-1, specified amount of real and reactive power are shared between the utility and the microgrid through the back-to-back converters. Mode-2 is invoked when the power that can be supplied by the distributed generators (DGs) in the microgrid reaches its maximum limit. In such a case, the rest of the power demand of the microgrid has to be supplied by the utility. An arrangement between DGs in the microgrid is proposed to achieve load sharing in both grid connected and islanded modes. The back-to-back converters also provide total frequency isolation between the utility and the microgrid. It is shown that the voltage or frequency fluctuation in the utility side has no impact on voltage or power in microgrid side. Proper relay-breaker operation coordination is proposed during fault along with the blocking of the back-to-back converters for seamless resynchronization. Both impedance and motor type loads are considered to verify the system stability. The impact of dc side voltage fluctuation of the DGs and DG tripping on power sharing is also investigated. The efficacy of the proposed control arrangement has been validated through simulation for various operating conditions. The model of the microgrid power system is simulated in PSCAD.
Personal control decreases narcissistic but increases non-narcissistic in-group positivity.

OBJECTIVE We examined the effects of control motivation on in-group positivity. Past research suggests that people compensate for low personal control by increasing support for social in-groups. We predicted that the effect of personal control on in-group positivity would depend on the type of in-group positivity. Low personal control should increase compensatory, narcissistic in-group positivity, whereas high personal control should increase secure, non-narcissistic in-group positivity. METHOD These hypotheses were tested in a cross-sectional survey (Study 1 N = 1,083, 54% female, Mage = 47.68), two experiments (Study 2 N = 105, 50% female, Mage = 32.05; Study 3 N = 154, 40% female, Mage = 29.93), and a longitudinal survey (Study 4 N = 398, 51% female, Mage = 32.05). RESULTS In all studies, personal control was negatively associated with narcissistic in-group positivity but positively associated with non-narcissistic in-group positivity. The longitudinal survey additionally showed that the positive relationship between personal control and non-narcissistic in-group positivity was reciprocal. Moreover, both types of in-group positivity differentially mediated between personal control and out-group attitudes: Narcissistic in-group positivity predicted negative attitudes, and non-narcissistic positivity predicted positive attitudes. CONCLUSIONS These findings highlight the role of individual motivation in fostering different types of in-group positivity and intergroup outcomes.
Protein Components of Mitochondrial DNA Nucleoids in Higher Eukaryotes*

Mitochondrial DNA (mtDNA) is not packaged in nucleosomal particles, but has been reported to associate with the mitochondrial inner membrane. Gentle lysis of Xenopus oocyte mitochondria with nonionic detergent liberates a nucleoprotein complex containing mtDNA associated with a previously characterized DNA binding partner, mitochondrial transcription factor A (mtTFA), as well as a series of inner membrane proteins identified by sequencing. More extensive detergent treatment stripped most of these proteins from the DNA, leaving a limited number of proteins in a nucleoid core. Sequencing of the major proteins retained in association with mtDNA revealed the expected mtDNA binding proteins, mtTFA and mitochondrial single-stranded DNA binding protein (mtSSB), as well as four proteins not previously reported to associate with mtDNA. These include adenine nucleotide translocator 1, the lipoyl-containing E2 subunits of pyruvate dehydrogenase and branched chain α-ketoacid dehydrogenase and prohibitin 2. The association of several of these proteins with mtTFA-containing mtDNA nucleoids was confirmed by immunoprecipitation.
Inside Job: Understanding and Mitigating the Threat of External Device Mis-Binding on Android

In this paper, we present the first study on this new security issue, which we call external Device Mis-Bonding or DMB, under the context of Bluetooth-enabled Android devices. Our research shows that this problem is both realistic and serious: oftentimes an unauthorized app can download sensitive user data from an Android device and also help the adversary to deploy a spoofed device that injects fake data into the original device’s official app on the phone. Specifically, we performed an in-depth analysis on four popular health/medical devices that collect sensitive user information and successfully built end-toend attacks that stealthily gathered sensitive user data and fed arbitrary information into the user’s health/medical account, using nothing but Bluetooth permissions and public information disclosed by the phone. Our further study of 68 relevant deviceusing apps from Google Play confirms that the vast majority of the devices on the market are vulnerable to this new threat. To defend against it, we developed the first OS-level protection, called Dabinder. Our approach automatically generates secure bonding policies between a device and its official app, and enforces them when an app attempts to establish Bluetooth connections with a device and unpair the phone from the device (for resetting the Bluetooth link key). Our evaluation shows that this new technique effectively thwarts the DMB attacks and incurs only a negligible impact on the phone’s normal operations.
Ultrapulse Carbon Dioxide Laser Ablation of Xanthelasma Palpebrarum: A Case Series

Context: Xanthelasma palpebrarum is the most common form of xanthomas. Albeit a benign entity, it is cosmetically disturbing and a frequently recurring dermatologic referral. Although the classical treatment option remains surgical excision, alternatively, chemical cauterization, cryosurgery and electrofulguration have all been tried in the past with mixed results. The use of laser systems such as carbon dioxide laser, Erb:YAG laser, Q-switched Nd:YAG laser, diode laser, pulsed dye laser and KTP laser have become popular in the treatment of these lesions. Recent literature suggests minimal pigmentary changes and scarring with the use of ultrapulse carbon dioxide laser treatment of these lesions. Aim: To study and evaluate the effectiveness of ultrapulse carbon dioxide laser ablation for treatment of xanthelasma palpebrarum. Materials and Methods: 10 patients presenting with bilateral xanthelasma palpebrarum, new and with recurrence were studied for results after a single treatment with ultrapulse carbon dioxide laser (10,600 nm; 100-200 Hz; 200-400 μsec). The follow-up time was 9 months. Results: All lesions were treatable with a single-laser treatment session. Two patients (20%) developed recurrence during the follow-up period. Side effects included post inflammatory hyperpigmentation in two patients (20%), but no visible scarring was observed. Conclusions: The ultrapulse carbon dioxide laser is an effective and safe therapeutic alternative in treatment of xanthelasma palpebrarum.
Preparation of mucosal nanoparticles and polymer-based inactivated vaccine for Newcastle disease and H9N2 AI viruses

Aim: To develop a mucosal inactivated vaccines for Newcastle disease (ND) and H9N2 viruses to protect against these viruses at sites of infections through mucosal immunity. Materials and Methods: In this study, we prepared two new formulations for mucosal bivalent inactivated vaccine formulations for Newcastle and Avian Influenza (H9N2) based on the use of nanoparticles and polymer adjuvants. The prepared vaccines were delivered via intranasal and spray routes of administration in specific pathogen-free chickens. Cell-mediated and humoral immune response was measured as well as challenge trial was carried out. In addition, ISA71 water in oil was also evaluated. Results: Our results showed that the use of spray route as vaccination delivery method of polymer and nanoparticles Montanide™ adjuvants revealed that it enhanced the cell mediated immune response as indicated by phagocytic activity, gamma interferon and interleukin 6 responses and induced protection against challenge with Newcastle and Avian Influenza (H9N2) viruses. Conclusion: The results of this study demonstrate the potentiality of polymer compared to nanoparticles adjuvantes when used via spray route. Mass application of such vaccines will add value to improve the vaccination strategies against ND virus and Avian influenza viruses.