Differential Sampling of AC Waveforms Based on a Commercial Digital-to-Analog Converter for Reference.

This paper introduces an innovative differential sampling technique for calibrating AC waveforms, leveraging a commercially available 16-bit digital-to-analog converter (DAC) as the reference standard. The novelty of this approach lies in its enhanced stability over traditional direct sampling methods, especially as the frequency of the AC waveform increases. Notably, this technique provides a cost-effective sampler alternative to the differential sampling methods that rely on a programmable Josephson voltage standard (PJVS). A critical aspect of this methodology is the precise measurement of the DAC's output voltage, for which a static measurement strategy is adopted to utilize the exceptional linearity and transfer accuracy of the Keysight 3458A (Santa Rosa, CA, USA) in its standard DCV mode. The differential sampling method has demonstrated good accuracy, achieving a near 1 µV/V agreement with a pulse-driven AC Josephson voltage standard (ACJVS) across a 40 Hz to 200 Hz frequency range. The method attained an expanded uncertainty (k = 2) of 1 part in 106 while measuring a 0.707107 VRMS sine wave at 50 Hz, showcasing its efficacy in precise AC waveform calibration.

[Analysis of NF1 gene variants in a Chinese pedigree and a sporadic patient with Neurofibromatosis type 1].

OBJECTIVE: To explore the genetic basis for a Chinese pedigree and a sporadic case with Neurofibromatosis type 1 (NF1). METHODS: Clinical data of the pedigree and the sporadic case were collected. Genomic DNA was extracted from peripheral venous blood samples and subjected to whole exome sequencing. Candidate variants were validated by Sanger sequencing and bioinformatic analysis. RESULTS: All patients from the pedigree were found to harbor a c.3251delC variant in exon 25 of the NF1 gene, whilst a c.4312_4314delGAA variant was found in exon 32 of the NF1 gene in the sporadic case. CONCLUSION: Variants of the NF1 gene may account for the occurrence of NF1 in this pedigree and sporadic case.

An improved electronic determination of the Boltzmann constant by Johnson noise thermometry.

Recent measurements using acoustic gas thermometry have determined the value of the Boltzmann constant, k, with a relative uncertainty less than 1 × 10-6. These results have been supported by a measurement with a relative uncertainty of 1.9 × 10-6 made with dielectric-constant gas thermometry. Together, the measurements meet the requirements of the International Committee for Weights and Measures and enable them to proceed with the redefinition of the kelvin in 2018. In further support, we provide a new determination of k using a purely electronic approach, Johnson noise thermometry, in which the thermal noise power generated by a sensing resistor immersed in a triple-point-of-water cell is compared to the noise power of a quantum-accurate pseudo-random noise waveform of nominally equal noise power. The experimental setup differs from that of the 2015 determination in several respects: a 100 Ω resistor is used as the thermal noise source, identical thin coaxial cables made of solid beryllium-copper conductors and foam dielectrics are used to connect the thermal and quantum-accurate noise sources to the correlator so as to minimize the temperature and frequency sensitivity of the impedances in the connecting leads, and no trimming capacitors or inductors are inserted into the connecting leads. The combination of reduced uncertainty due to spectral mismatches in the connecting leads and reduced statistical uncertainty due to a longer integration period of 100 d results in an improved determination of k = 1.380 649 7(37) × 10-23 J K-1 with a relative standard uncertainty of 2.7 × 10-6 and a relative offset of 0.89 × 10-6 from the CODATA 2014 recommended value. The most significant terms in the uncertainty budget, the statistical uncertainty and the spectral-mismatch uncertainty, are uncorrelated with the corresponding uncertainties in the 2015 measurements.

Endoplasmic reticulum stress-induced autophagy determines the susceptibility of melanoma cells to dabrafenib.

Melanoma is one of the deadliest skin cancers and accounts for most skin-related deaths due to strong resistance to chemotherapy drugs. In the present study, we investigated the mechanisms of dabrafenib-induced drug resistance in human melanoma cell lines A375 and MEL624. Our studies support that both endoplasmic reticulum (ER) stress and autophagy were induced in the melanoma cells after the treatment with dabrafenib. In addition, ER stress-induced autophagy protects melanoma cells from the toxicity of dabrafenib. Moreover, inhibition of both ER stress and autophagy promote the sensitivity of melanoma cells to dabrafenib. Taken together, the data suggest that ER stress-induced autophagy determines the sensitivity of melanoma cells to dabrafenib. These results provide us with promising evidence that the inhibition of autophagy and ER stress could serve a therapeutic effect for the conventional dabrafenib chemotherapy.