Gain-of-Function Mutations R249C and S250C in Complement C2 Protein Increase C3 Deposition in the Presence of C-Reactive Protein

The impairment of the alternative complement pathway contributes to rare kidney diseases such as atypical hemolytic uremic syndrome (aHUS) and C3 glomerulopathy (C3G). We recently described an aHUS patient carrying an exceptional gain-of-function (GoF) mutation (S250C) in the classical complement pathway component C2 leading to the formation of hyperactive classical convertases. We now report the identification of the same mutation and another C2 GoF mutation R249C in two other patients with a glomerulopathy of uncertain etiology. Both mutations stabilize the classical C3 convertases by a similar mechanism. The presence of R249C and S250C variants in serum increases complement-dependent cytotoxicity (CDC) in antibody-sensitized human cells and elevates deposition of C3 on ELISA plates coated with C-reactive protein (CRP), as well as on the surface of glomerular endothelial cells. Our data justify the inclusion of classical pathway genes in the genetic analysis of patients suspected of complement-driven renal disorders. Also, we point out CRP as a potential antibody-independent trigger capable of driving excessive complement activation in carriers of the GoF mutations in complement C2

An analysis and evaluation of the WeFold collaborative for protein structure prediction and its pipelines in CASP11 and CASP12

Every two years groups worldwide participate in the Critical Assessment of Protein Structure Prediction (CASP) experiment to blindly test the strengths and weaknesses of their computational methods. CASP has significantly advanced the field but many hurdles still remain, which may require new ideas and collaborations. In 2012 a web-based effort called WeFold, was initiated to promote collaboration within the CASP community and attract researchers from other fields to contribute new ideas to CASP. Members of the WeFold coopetition (cooperation and competition) participated in CASP as individual teams, but also shared components of their methods to create hybrid pipelines and actively contributed to this effort. We assert that the scale and diversity of integrative prediction pipelines could not have been achieved by any individual lab or even by any collaboration among a few partners. The models contributed by the participating groups and generated by the pipelines are publicly available at the WeFold website providing a wealth of data that remains to be tapped. Here, we analyze the results of the 2014 and 2016 pipelines showing improvements according to the CASP assessment as well as areas that require further adjustments and research

Experimental verification of parameters and characteristics of robotic rangefinder

Zróżnicowane wyposażenie autonomicznego robota mobilnego (ARM) w sensory, w gruntowny sposób wpływa na proces sterowania tymże obiektem. W artykule dokonano przeglądu czujników opcjonalnie stosowanych w ARM. Szczegółowym badaniom poddano dalmierze GP2Y0A0YK pracujące w paśmie podczerwieni. Opracowano ich tor pomiarowy. W kolejnym kroku zbadano charakterystyki statyczne pracy sensorów i porównano z danymi katalogowymi producenta. Celem badań dynamicznych zainstalowano dwa czujniki na doświadczalnej platformie mobilnej. Sprawdzono ich wzajemne zakłócanie się oraz odpowiedź czujnika na pojawiające się nieruchome i ruchome przeszkody.Sensor equipment of autonomous mobile robot (AMR) significantly influence control process of robot. In the paper authors have surveyed sensors that can be applied to AMR. Infrared rangefinder GP2Y0A0YK is submitted to detailed examination. Measurement path was designed. In next step, static characteristics are examined and compared with those from data sheet given by producer. For dynamical research two sensors are installed on experimental mobile platform. Measurement signals and their mutual interfering for stationary and mobile obstacles were examined

Cognitive architectures survey of methods and implementations

Autonomiczne roboty mobilne są wykorzystywane w wielu nowych zastosowaniach. Zastosowania te wymagają autonomii trudnej do przewidzenia przez projektanta systemów robotycznych. Stąd też potrzeba zastosowania architektur sterowania zwanych kognitywnymi. Architektury kognitywne mają zaimplementowane mechanizmy, dzięki którym potrafią autonomicznie rozwiązywać nowe problemy oraz uczyć się na podstawie rozwiązań problemów już napotkanych. W artykule omówiono trzy różne architektury kognitywne oraz implementacje dwóch z nich oraz dokonano symulacji.Autonomous mobile robots are used in different new applications. These applications put different requirements on the autonomy tasks of robotic systems. The more advances in autonomy solutions are connected with these cognitive architectures. Cognitive architectures have build-in mechanisms, which can cope with new problems arriving in uncertain environments. In the paper three different cognitive architectures, implementations and simulation of them have been described and compared

HIL investigations of extended UAV on-board controller

The paper presents the testing process dedicated to the unmanned aerial vehicle flight controller. The quality of performance of stabilization and navigation tasks has been investigated not during standard in-flight tests, but in the hardware in the loop (HIL) simulation environment. The virtual flight was controlled by the real autopilot. The artificial flight parameters were generated and autopilot uses them as in real flight conditions. Such approach saves time and is economically justified, because of the lack of hardware losses during the failed flight tests

Integration and in-field gains selection of flight and navigation controller for remotely piloted aircraft system

In the paper the implementation process of commercial flight and navigational controller in own aircraft is shown. The process of autopilot integration were performed for the fixed-wing type of unmanned aerial vehicle designed in high-wing and pull configuration of the drive. The above equipment were integrated and proper software control algorithms were chosen. The correctness of chosen hardware and software solution were verified in ground tests and experimental flights. The PID controllers for longitude and latitude controller channels were selected. The proper deflections of control surfaces and stabilization of roll, pitch and yaw angles were tested. In the next stage operation of telecommunication link and flight stabilization were verified. In the last part of investigations the preliminary control gains and configuration parameters for roll angle control loop were chosen. This enable better behavior of UAV during turns. Also it affected other modes of flight such as loiter (circle around designated point) and auto mode where the plane executed a pre-programmed mission

Multirotor Motor Failure Detection with Piezo Sensor

Failure detection of Unmanned Aerial Vehicle (UAV) motors and propulsion systems is the most important step in the implementation of active fault-tolerant control systems. This will increase the reliability of unmanned systems and increase the level of safety, especially in civil and commercial applications. The following paper presents a method of motor failure detection in the multirotor UAV using piezo bars. The results of a real flight, in which the failure of the propulsion system caused the crash of a hybrid VTOL UAV, were presented and analyzed. The conclusions drawn from this flight led to the development of a lightweight, simple and reliable sensor that can detect a failure of the UAV propulsion system. The article presents the outcomes of laboratory tests concerning measurements made with a piezo sensor. An extensive analysis of the obtained results of vibrations recorded on a flying platform arm with a propulsion system is presented, and a methodology for using this type of data to detect failures is proposed. The article presents the possibility of using a piezoelectric sensor to record vibrations on the basis of which it is possible to detect a failure of the UAV propulsion system

An identification of electric drive model for quadrotor

Niniejsza praca przedstawia zagadnienia z zakresu identyfikacji modelu dynamicznego napędu dedykowanego dla bezzałogowego aparatu latającego (w skrócie BAL) pionowego startu typu VTOL (z ang. vertical take-off and landing) na przykładzie konfiguracji śmigłowca czterowirnikowego. Dokonano przeglądu rozwiązań konstrukcyjnych oraz trendów w rozwoju powietrznych statków bezpilotowych na przestrzeni lat. Zaprezentowano stanowisko doświadczalne do pomiaru zmian siły ciągu w dziedzinie czasu. Wyznaczono i przeanalizowano charakterystykę statyczną oraz dynamiczną wybranego zespołu napędowego. Zidentyfikowano model dynamiczny napędu.In the paper ARX method is use for the identification of electric drive (propeller plus motor) mathematical model. Drive is dedicated to unmanned aerial vehicle ( UAV ) of vertical take-off and landing (VTOL) type, basing of four-rotor configuration drone helicopter. The survey on constructional solutions and trends in development of UAVs is presented. A test platform to measure changes of thrust in time domain is described. Static and dynamic characteristics of chosen propulsion unit are shown. Dynamical model of drive was identified