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Deubiquitinases (DUBs) are enzymes that control the stability, interactions or localization of most cellular proteins by removing their ubiquitin modification. In recent years, some DUBs, such as USP7, USP9X and USP10, have been identified as promising therapeutic targets in hematological malignancies. Importantly, some potent inhibitors targeting the oncogenic DUBs have been developed, showing promising inhibitory efficacy in preclinical models, and some have even undergone clinical trials. Different DUBs perform distinct function in diverse hematological malignancies, such as oncogenic, tumor suppressor or context-dependent effects. Therefore, exploring the biological roles of DUBs and their downstream effectors will provide new insights and therapeutic targets for the occurrence and development of hematological malignancies. We summarize the DUBs involved in different categories of hematological malignancies including leukemia, multiple myeloma and lymphoma. We also present the recent development of DUB inhibitors and their applications in hematological malignancies. Together, we demonstrate DUBs as potential therapeutic drug targets in hematological malignancies.

Background PYGL mutations can cause liver phosphorylase deficiency, resulting in a glycogenolysis disorder, namely, glycogen storage disease (GSD) VI. The disease is rarely reported in the Chinese population. GSD VI is mainly characterized in untreated children by hepatomegaly, growth retardation and elevated liver transaminases. Case presentation In this study, we report two GSD VI patients with growth retardation and abnormal liver function. There was no obvious hepatomegaly for one of them. Whole exome sequencing (WES) combined with copy number variation analysis was performed. We found a novel homozygous gross deletion, c.1621-258_2178-23del, including exons 14–17 of PYGL in patient 1. The exons 14–17 deletion of PYGL resulted in an in-frame deletion of 186 amino acids. Compound heterozygous mutations of PYGL were identified in patient 2, including a novel missense mutation c.1832C > T/p.A611V and a recurrent nonsense mutation c.280C > T/p.R94X. After treatment with uncooked cornstarch (UCS) 8 months for patient 1 and 13 months for patient 2, the liver transaminases of both patients decreased to a normal range and their stature was improved. However, patient 1 still showed mild hypertriglyceridemia. Conclusions We describe two GSD VI patients and expand the spectrum of PYGL mutations. Patient 1 in this study is the first GSD VI case that showed increased transaminases without obvious hepatomegaly due to a novel homozygous gross deletion of PYGL identified through WES.

The symmetrical cell model is widely used to study the residual stress induced by shot peening. However, the correlation between the predicted residual stresses and the shot peening coverage, which is a big challenge for the researchers of the symmetrical cell model, is still not established. Based on the dynamic stresses and the residual stresses outputted from the symmetrical cell model, the residual stresses corresponding to full coverage are evaluated by normal distribution analysis. The predicted nodal dynamic stresses with respect to four corner points indicate that the equi-biaxial stress state exists only for the first shot impact. Along with the increase of shot number, the interactions of multiple shot impacts make the fluctuation of the nodal dynamic stresses about an almost identical value more and more obvious. The mean values and standard deviations of the residual stresses gradually tend to be stable with the increase of the number of shot peening series. The mean values at each corner point are almost the same after the third peening series, which means that an equi-biaxial stress state corresponding to the full coverage of shot peening is achieved. Therefore, the mean values of the nodal residual stresses with respect to a specific transverse cross-section below the peened surface can be used to correlate the measured data by X-ray. The predicted residual stress profile agrees with the experimental results very well under 200% peening coverage. An effective correlation method is proposed for the nodal residual stresses predicted by the symmetrical cell model and the shot peening coverage.

The equipment performance monitoring program is designed to provide guidance for monitoring the performance of equipment, with an aim to enable engineers to identify hidden hazards as early as possible and take corresponding corrective actions to achieve the purpose of equipment reliability management of nuclear power plant. The nuclear power plant realizes monitoring, control and protection of the whole plant through I&C equipment, by which to ensure the safe, reliable and economic operation of the nuclear power plant. In this paper, a set of performance monitoring program process special for I&C equipment of nuclear power plant is developed. And the RGL system (rod control system and rod position system) of a nuclear power plant is taken as an example to illustrate, analyze and summarize, which lays a foundation for nuclear power plant to carry out equipment reliability management.

The Brillouin distributed sensing system is sensitive to the crossover between strain and temperature. The temperature and strain can be measured simultaneously through decoupling. However, there is no suitable method to calibrate and trace the strain measurement. Therefore, a method of calibrating fiber strain by grating ruler is proposed, the decoding circuit of grating ruler is designed, and the calibration device of optical fiber strain is built. The experimental results show that the uncertainty reaches 5.90 μm (k = 2) in the range of 0 ∼ 1000 μm, and the measurement of optical fiber strain can be traced back to the national length standard.

An investigation of the interaction of a vortex ring colliding with a coaxially aligned aperture, which extends the classical problem of a vortex ring impacting a solid wall, examines the behaviors of near-wall vortices in the proximity of a structural discontinuity (aperture tip).

The traditional temperature measurement system used the ratio of stokes light and anti-stokes light to demodulate the temperature, and there was an error when the ratio used directly. The propagation speed of stokes light and anti-stokes light in the same fiber was different because of dispersion effect. The two kinds of reflected light were obtained by photoelectric converter at different time when the scattering occurred at the same place. Therefore, there was a time warping between the two signals after data acquisition. This phenomenon would lead to measurement error in the process of demodulating temperature. A new method of error correction for the distributed temperature measurement system was used to eliminate the signal dislocation by correcting the stokes signal. In the absence of correction, the antistokes signal peak and the stokes signal peak occurred in different place, which leaded to errors in the process of demodulating temperature. This correcting method was used. The demodulation error was reduced from 2.3 °C to 1.2 °C. The shortest time was 4.96s when detected temperature rise rate was not less than 5 °C/min in the case of 10km fiber carried by this system. It was showed that this technique could improve the performance of the traditional distributed fiber Raman temperature measurement system, and it would have a good aspect.

In order to monitor the early fire situation caused by coal spontaneous combustion or cable aging, cable heated, cable overcurrent and other reasons, a dual channel and fast response temperature detection system is designed based on the Raman scattering principle in which anti-stokes signal in back Raman scattering is sensitive to temperature. The performance of the temperature detection system is obtained through experiments. The experimental data shows that the maximum error of temperature measurement is 0.7°C, the stability is 1°C in 30 minutes when both channels carry 2.5km sensing fiber, single channel response time is less than 17s and position-calculated error is less than 2m. This system would have a good application prospect in distributed fire detection and early warning under mine.

Temperature and strain measurement accuracy of Brillouin distributed optical fiber sensor are easily influenced by the performance of laser light source. A wavelength/power DFB semiconductor laser light source with high-stability used for Brillouin Fiber Sensing is designed. The laser light source works with constant-current drive circuit and temperature control circuit, which precisely controls drive current and operating temperature of the DFB semiconductor laser and makes the wavelength and optical power under control. The results show that: (1) the optical wavelength increases about 0.1nm and the power reduces about 0.05dBm when the temperature increased by 1°C . (2) The maximum drift of wavelength is 0.012nm and the maximum drift of optical power is 0.05dBm (0.0014mW) at 25 °C within one hour. The laser light source can completely meet the demand for Brillouin distributed optical fiber sensing.

A high-precision temperature-controlled narrow band-stop fiber Bragg grating (FBG) filter and light source self-calibration technique are proposed for application in the Brillouin optical time domain reflectometer (BOTDR) sensor system. With the proposed application, the BOTDR sensor system maintains good long-term stability and temperature precision through the reduction of the center wavelength drift in the FBG filters and corresponding decrease in the changes in light intensity. The experiment result shows that temperature precision of 1 °C and temperature stability of 0.7 °C can be achieved in a temperature sensor over a range of 8 km.

A dual-source distributed optical fiber sensor system with combined Raman and Brillouin scatterings is designed for simultaneous temperature and strain measurements. The optimal Raman and Brillouin signals can be separately obtained by adjusting the powers of the two sources using an optical switch. The temperature and strain can be determined by processing the optimal Raman and Brillouin signals. The experimental result shows that 1.7 oC temperature resolution and 60-\mu \varepsilon strain resolution can be achieved at a 24.7-km distance.

The signal-to-noise ratio (SNR) enhancement using polarization scrambler, narrowband-stop grating and cumulative average processing in Brillouin optical time domain reflectometer (BOTDR) is studied experimentally. Compared to the result without these methods, it is confirmed that these techniques can provide the SNR enhancement in our experiment. This makes it possible to perform a better detection threshold and resolution. The experiment result shows that a temperature resolution of 2°C with a spatial resolution of 3 m is achieved on our BOTDR sensor system.

Structural health monitoring (SHM) can be understood as the integration of sensing intelligence and possibly also actuation devices to allow the structure loading and damaging conditions to be recorded, analyzed, localized and predicated. SHM sensing requirements are very well suited for fiber optic technology. The fiber Bragg grating (FBG) sensor is a promising sensor of SHM for many applications because of its good optical properties, small size, low insertion loss, antiRF, anti-electromagnetic interference, breakdown voltage, radiation-resistant, light weight, small and large signal bandwidth. There also are reports of the application of FBG sensors for SHM of large industrial and civil works such as dams, bridges [1−2] , aero plane [3] , and tunnels [4,5] . The development of ship’s SHM takes advantage of multidisciplinary work that is being done, and most of the applications reported are about ship hull monitoring systems [6,7] . Previous studies mostly focus on the measurement of static stress [8] . For a modern ship, the thruster is a key part of the propulsion machinery, and the stability of its related equipments has a great influence on the propulsion efficiency of the ship. Electric resistance strain sensors are difficult to be used for the stress measurement for the thruster because of the electromagnetic disturbance and other complicated environment. Low-speed FBG sensors cannot meet the requirements due to the propulsion machinery’s 100 Hz arising vibration frequency. The high-speed FBG sensing system can be used to measure dynamic stress and achieve the goal of safety monitoring in advanced ships. In this letter, the application of a high-speed FBG sensing system for SHM of modern ships is proposed. We apply this sensing system to give on-site tests to an advanced ship. Strain distribution of the bases and supporting frames of its large turbine thrusters are measured both onshore and in the sea when it runs at different work conditions. The maximum natural frequency of this measured structure is 335 Hz. To test all the monitoring points synchronously, an 8-channel senor system is applied, and the data acquisition frequency was 1000 Hz. Most monitoring points are set with single-line sensors for unilateral stress, and the others are set with triple directions sensors to measure shear stress. Temperature compensation is taken with additional FBGs to eliminate the effect of cross-sensitivity between temperature and strain on FBG sensors. As for every single monitoring point, the max and average stress-gas turbine power factor (GTPF): N/Ne curves are analyzed. Then we synthesize all the results from three-time onshore tests and acquire the static stress-GTPF curves. Results from the test at sea are discussed together, thus we can accomplish completely prediction of the dynamic stress-GTPF curve when we did not obtain dynamic data in every work condition because of some objective reason. We examine the vibration amplitude of the wave length of signal light from each specific monitoring point under the same work condition for all tests and find it barely affected by the environment factors, unlike the absolute wave length. Those experimental results are accordant with mechanical theories, and the final reports have obtained clients’ approval. FBGs are periodic alterations in the index of refraction of the fiber core, and only the specified wavelength (Bragg wavelength) related to its grating period is reflected for an input light wave form a broadband source.