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This paper addresses a tracking control problem with prespecified accuracy for uncertain switching nonlinear systems under arbitrary switching with periodically time-varying parameters. To approximate the unknown nonlinear functions and unknown periodically time-varying parameters, the radial basis function neural network and Fourier series expansion are introduced, respectively. Compared with the previous results of unknown nonlinear switching system approximation, the upper bounds of the approximation errors are considered for the first time. And then, a new adaptive NN control scheme is constructed by using backstepping technique and common Lyapunov function theory. It can be proved that all the signals in the closed-loop system are semi-globally uniformly ultimately bounded and the tracking error converges to the prescribed neighborhood of zero. Two examples are provided to verify the feasibility and advantages of the proposed approach in this paper.

Lethal neonatal rigidity and multifocal seizure syndrome (RMFSL) is caused by variants in BRAT1 (BRCA1-associated protein required for ATM activation-1). However, the molecular mechanism of RMFSL is still unclear.An RMFSL infant was recruited and the peripheral blood samples from his trio-family were collected. The genomic DNA was extracted, and then the whole-exome sequencing was performed. The expression of BRAT1 was analyzed by Western blotting. The subcellular localization of BRAT1 and MitoSOX (mitochondrial superoxide level) was investigated by confocal microscopy. The RNA samples were obtained from transfected cells, and then the RNA sequencing was performed.In this study, a novel homozygous BRAT1 variant c.233G C with amino acid change of R with P at residue 78 (R78P) was identified. This variant altered the peptide structure and subcellular localization, as well as the expression in vitro. However, R78P did not alter the ability of BRAT1 to downregulate MitoSOX in mitochondria. Meanwhile, R78P BRAT1 was positively correlated with temporal lobe epilepsy, autosomal recessive primary microcephaly, defective/absent horizontal voluntary eye movements, and neuron apoptotic process as indicated by gene set enrichment analysis (GSEA).The BRAT1 variant spectrum has been expanded, which will be helpful for genetic counseling. We also explored the molecular mechanism altered by R78P, which will provide a better understanding of the pathogenesis of RMFSL.The detailed course of an infant with lethal neonatal RMFSL was depicted. A novel disease-causing variant R78P in BRAT1 for lethal neonatal RMFSL was identified. R78P led to reduced BRAT1 expression and nuclear localization in vitro. R78P did not alter the ability of BRAT1 to downregulate MitoSOX in the mitochondria. The variant R78P in BRAT1 was positively correlated with temporal lobe epilepsy, autosomal recessive primary microcephaly, defective/absent horizontal voluntary eye movements, and neuron apoptotic process as indicated by GSEA.

This paper focuses on asynchronous periodic sampling consensus for leader-following second-order multi-agent systems with event-triggered mechanism for the first time. A distributed control protocol is proposed to improve consistency and to reduce data transmission. We prove that the proposed algorithm is exponentially convergent if the topology contains a directed spanning tree. We acquire the consensus in two situations with the proposed controllers. (i) In the first situation, the positions converge to a nonzero constant and the velocities converge to zero when the velocity of the leader is zero; (ii) In the second situation, the errors of the positions and velocities between the followers and the virtual leader converge to a small region containing 0 when the velocity of the leader is a nonzero constant or time-varying. Finally, a numerical example with three cases is given to illustrate the effectiveness of the proposed asynchronous periodic sampling control method

Background Combined oxidative phosphorylation deficiency 26 (COXPD26) is an autosomal recessive disorder characterized by early onset, developmental delay, gastrointestinal dysfunction, shortness of breath, exercise intolerance, hypotonia and muscle weakness, neuropathy, and spastic diplegia. This disease is considered to be caused by compound heterozygous mutations in the TRMT5 gene. Case presentation In this study, we report a female child with COXPD26 manifesting as shortness of breath, gastrointestinal dysmotility, severe developmental delay, muscle hypotonia and weakness, exercise intolerance, renal and hepatic defects, and recurrent seizures with spastic diplegia. Interestingly, the hepatic feature was first observed in a COXPD26 patient. Medical exome sequencing with high coverage depth was employed to identify potential genetic variants in the patient. Novel compound heterozygous mutations of the TRMT5 gene were detected, which were c.881A>C (p.E294A) from her mother and c.1218G>C (p.Q406H) and c.1481C>T (p.T494M) from her father. Conclusion The newly emerged clinical features and mutations of this patient provide useful information for further exploration of genotype–phenotype correlations in COXPD26.