Prediction of binding affinity and enthalpy of CB7 with alkaloids by attach-pull-release molecular dynamics simulations study.

Host-guest complex has attracted much attention because of their fantastic capability. Accurate prediction of their binding affinity and enthalpy is essential to the rational design of guest molecules. The attach-pull-release (APR) method proposed by Henriksen et al.

Distributed Formation Control of Multi-Robot Systems with Path Navigation via Complex Laplacian.

This paper focuses on the formation control of multi-robot systems with leader-follower network structure in directed topology to guide a system composed of multiple mobile robot agents to achieve global path navigation with a desired formation. A distributed linear formation control strategy based on the complex Laplacian matrix is employed, which enables the robot agents to converge into a similar formation of the desired formation, and the size and orientation of the formation are determined by the positions of two leaders. Additionally, in order to ensure that all robot agents in the formation move at a common velocity, the distributed control approach also includes a velocity consensus component.

An adaptive collision avoidance strategy for autonomous vehicle under various road friction and speed.

When the autonomous vehicle (AV) is under various road friction and speed, emergency collision avoidance is extremely difficult. In this situation, the AV may encounter severe understeering problem, so it is hard to achieve collision avoidance, even under the control of active safety system. To tackle this problem, an adaptive collision avoidance strategy (ACAS) is proposed for AV under various road friction and speed.

Catalytic activity in vitro of the human protein kinase ASK1 mutants: Experimental and molecular simulation study.

Kinases have become an important class of targets for drug discovery since the milestone approval of imatinib in 2001. Although a great success has been achieved for targeting kinases with over 70 inhibitors approved by the FDA, it is inevitable that drug resistance would emerge during treatment. Thus, assessment of the kinase mutations is an essential issue for the development of the next generation inhibitors.

Finite-Time Pinning Synchronization Control for T-S Fuzzy Discrete Complex Networks with Time-Varying Delays via Adaptive Event-Triggered Approach.

This paper is concerned with the adaptive event-triggered finite-time pinning synchronization control problem for T-S fuzzy discrete complex networks (TSFDCNs) with time-varying delays. In order to accurately describe discrete dynamical behaviors, we build a general model of discrete complex networks via T-S fuzzy rules, which extends a continuous-time model in existing results. Based on an adaptive threshold and measurement errors, a discrete adaptive event-triggered approach (AETA) is introduced to govern signal transmission.

Adaptive fractional-order non-singular terminal sliding mode control based on fuzzy wavelet neural networks for omnidirectional mobile robot manipulator.

This paper studies a novel adaptive fractional-order non-singular terminal sliding mode (FO-NTSM) control strategy for omnidirectional mobile robot manipulator (OMRM) with unknown parameters and external disturbances. Firstly, we adopt the fuzzy wavelet neural networks (FWNNs) to estimate the dynamic uncertainty of the OMRM because it has superior function approximation capability. Secondly, we design the adaptive NTSM controller to attenuate external disturbances by virtue of adjusting the weights of the FWNNs online.

Incomplete differentiation-based improved adaptive backstepping integral sliding mode control for position control of hydraulic system.

In the hydraulic systems, the non-structural uncertainties such as the nonlinear friction will reduce the tracking accuracy for the hydraulic servo system. In this paper, an incomplete differential-based improved adaptive backstepping integral sliding mode control (ID-BIABISMC) is proposed to realize the position control for the hydraulic servo system based on the friction compensation. The backstepping-based control being integrated the integral sliding mode surface-based sliding mode control with the friction compensation are used to solve the problem of non-structural uncertainty of the hydraulic system.

Nuclear Factor-κB Pathway Mediates the Molecular Pathogenesis of LMNA-Related Muscular Dystrophies.

LMNA-related muscular dystrophies are caused by mutations of the LMNA gene. Inflammatory changes and cellular apoptosis are significant pathological findings in the muscle cells of these patients. We aimed to investigate the roles of nuclear factor-κB (NF-κB) mediated inflammation as a molecular mechanism for the pathogenesis of LMNA-related muscular dystrophies.

Clinical spectrum and genetic variations of -related muscular dystrophies in a large cohort of Chinese patients.

Background: -related muscular dystrophy is caused by mutations in gene. We aimed to identify genetic variations and clinical features in a large cohort of Chinese patients with mutations in an attempt to establish genotype-phenotype correlation.

Methods: The clinical presentations of patients with -related muscular dystrophy were recorded using retrospective and prospective cohort study.

MECP2 mutation spectrum and its clinical characteristics in a Chinese cohort.

The dysfunction of methyl-CpG-binding protein 2 (MeCP2) is associated with several neurological disorders, of which Rett syndrome (RTT) is the most prominent. This study focused on a Chinese patient cohort with MECP2 mutations, and analyzed the characteristics of these mutations and their clinical manifestations. In total, 666 patients were identified with 126 different MECP2 mutations, including 22 novel mutations.

A splice site mutation c.1251G>A of ISPD gene is a common cause of congenital muscular dystrophy in Chinese patients.

The predicted synonymous mutation c.1251G>A of ISPD (NM_001101426.3) is a hot spot causing exon 9 skipping in five patients.

CHD2-related epilepsy: novel mutations and new phenotypes.

The aim of this report was to refine the genotypes and phenotypes of chromodomain helicase DNA-binding protein 2 (CHD2)-related epilepsy. Seventeen patients with CHD2 mutations were enrolled. CHD2 mutations were identified by application of next-generation sequencing of epilepsy or whole exome sequencing.

Rett and Rett-like syndrome: Expanding the genetic spectrum to KIF1A and GRIN1 gene.

Background: This study aimed to investigate the new genetic etiologies of Rett syndrome (RTT) or Rett-like phenotypes.

Methods: Targeted next-generation sequencing (NGS) was performed on 44 Chinese patients with RTT or Rett-like phenotypes, in whom genetic analysis of MECP2, CDKL5, and FOXG1 was negative.

Results: The detection rate was 31.

The Efficacy of Ketogenic Diet in 60 Chinese Patients With Dravet Syndrome.

To evaluate the efficacy and safety of ketogenic diet (KD) in patients with Dravet syndrome (DS). 60 DS patients receiving treatment of KD for more than 12 weeks from 2009 to 2018 were analyzed retrospectively. Modified Johns Hopkins protocol was used to initiate KD.

Infantile epilepsy with multifocal myoclonus caused by TBC1D24 mutations.

Purpose: To summarize the clinical features and neuroimaging changes of epilepsy associated with TBC1D24 mutations.

Methods: Genetic testing was conducted in all epilepsy patients without acquired risk factors for epilepsy. Epilepsy patients identified with TBC1D24 compound heterozygous mutations by next-generation sequencing (NGS) epilepsy panel or whole exome sequencing (WES) were enrolled.

Congenital muscular dystrophies in China.

Congenital muscular dystrophies (CMDs) are clinically and genetically heterogeneous conditions. We launched a nationwide study to determine the frequency of CMD in the Chinese population and assess the status of diagnosis and disease management for CMD in China. Cases were chosen from databases in 34 tertiary academic hospitals from 29 first-level administrative divisions (provinces, municipalities, autonomous regions, and special administrative regions), and medical records were reviewed to confirm the diagnoses.

De novo SCN1A, SCN8A, and CLCN2 mutations in childhood absence epilepsy.

This study aimed to identify monogenic mutations from Chinese patients with childhood absence epilepsy (CAE) and summarize their characteristics. A total of 100 patients with CAE were recruited in Peking University First Hospital from 2005 to 2016 and underwent telephone and outpatient follow-up review. We used targeted disease-specific gene capture sequencing (involving 300 genes) to identify pathogenic variations for these patients.

ATP1A3 mosaicism in families with alternating hemiplegia of childhood.

Alternating hemiplegia of childhood (AHC) is a rare and severe neurodevelopmental disorder characterized by recurrent hemiplegic episodes. Most AHC cases are sporadic and caused by de novo ATP1A3 pathogenic variants. In this study, the aim was to identify the origin of ATP1A3 pathogenic variants in a Chinese cohort.

Correction: Genomic mosaicism in the pathogenesis and inheritance of a Rett syndrome cohort.

The second author Jiarui Li is now listed as a co-first author according to her contribution to this paper. The list of authors who contributed equally now reads: Qingping Zhang, Xiaoxu Yang, Jiaping Wang, and Jiarui Li. This has now been corrected in both the PDF and HTML versions of the Article.

Gene mutational analysis in a cohort of Chinese children with unexplained epilepsy: Identification of a new KCND3 phenotype and novel genes causing Dravet syndrome.

Purpose: This study aimed to investigate the genetic etiology of epilepsy in a cohort of Chinese children.

Methods: Targeted next-generation sequencing (NGS) was performed for 120 patients with unexplained epilepsy, including 71 patients with early-onset epileptic encephalopathies, and 16 patients with Dravet syndrome (including three patients with a Dravet-like phenotype) but without SCN1A pathogenic variants.

Results: Pathogenic variants of 14 genes were discovered in 22 patients (18%).

Genomic mosaicism in the pathogenesis and inheritance of a Rett syndrome cohort.

Purpose: To determine the role of mosaicism in the pathogenesis and inheritance of Rett and Rett-like disorders.

Methods: We recruited 471 Rett and Rett-like patients. Panel-sequencing targeting MECP2, CDKL5, and FOXG1 was performed.

Novel MEF2C point mutations in Chinese patients with Rett (-like) syndrome or non-syndromic intellectual disability: insights into genotype-phenotype correlation.

Background: MEF2C (Myocyte-specific enhancer factor 2C) has been associated with neurodevelopmental disorders. This study aimed at delineating the clinical profiles of MEF2C gene mutations.

Methods: In total, 112 Chinese patients with intellectual disability (ID) were recruited, including 44 patients presented with Rett syndrome (RTT) or RTT-like syndrome, and 68 patients with non-syndromic ID.

Large De Novo Microdeletion in Epilepsy with Intellectual and Developmental Disabilities, with a Systems Biology Analysis.

Epilepsy is one of the most common complex neurological diseases. It is frequently associated with intellectual and developmental disabilities (ID/DD). In recent years, copy number variation (CNV), especially microdeletion, was proven to be a potential key factor of genetic epilepsy.