A Triple‐Shape Memory Material via Thermal Responsive Behavior of Liquid Crystalline Network Incorporating Main‐Chain/Side‐Chain LC Units

In the last several years, multiple‐shape memory liquid crystalline networks (LCNs) have received more and more attention due to the basic theoretical research on them and their wide potential applications. In this article, a novel main‐chain/side‐chain liquid crystalline monomer and its corresponding polymer networks based on the thiol‐ene click reaction are reported. Properties of the synthesized liquid crystalline monomer are well studied with nuclear magnetic resonance (NMR), Fourier transform infrared (FTIR) spectra, differential scanning calorimetry (DSC), and polarized optical microscopy (POM). The as‐prepared free‐standing LCN films are investigated well by FTIR, DSC, POM, and X‐ray diffraction (XRD), which show them having good liquid crystalline properties. Tensile test and dynamical mechanical analysis (DMA) results indicate the LCN films have excellent thermal mechanical properties. By adjusting the crosslinking densities, LCN films exhibit two thermal transition temperatures (T_g and T_NI) that can be utilized to trigger the triple‐shape memory behaviors. The cyclic thermal mechanical analysis conducted by DMA reveals that LCN films exhibit good triple‐shape memory properties with high‐shape fixity ratio (R_f (S1→S2) is 99.2% and R_f (S2→S3) is 99.3%) and shape recovery ratio (R_r (S3→S2) is 92.4% and R_r (S2→S1) is 98.5%).     

Rapamycin attenuates Tc1 and Tc17 cell responses in cigarette smoke-induced emphysema in mice

Inflammation Research - Chronic exposure to cigarette smoke promotes airway inflammation and emphysema accompanied by enhanced CD8+ interferon (IFN)-γ+ T(Tc1) and CD8+ interleukin (IL)-17+...     

TNF-α/calreticulin dual signaling induced NLRP3 inflammasome activation associated with HuR nucleocytoplasmic shuttling in rheumatoid arthritis

Inflammation Research - The present study was undertaken to validate whether TNF-α and calreticulin (CRT) serve as dual signaling to activate nucleotide-binding oligomerization domain-,...     

An emerging novel virus: Atypical porcine pestivirus (APPV)

Emerging porcine pestivirus diseases frequently challenge prevention and control strategies in the swine industry. Over the past decade, a few novel pestiviruses have been identified in pigs. This article focuses on the recently emerging atypical porcine pestivirus (APPV) that potentially threatens global swine herd health security. The virus was first identified in 2016, in the United States and thereafter, accumulated evidence shows that it is currently distributed in three continents. The clinical presentation of APPV‐infected pigs is characterized by congenital tremor (CT) type A‐II in piglets, while adult pigs may become persistent carriers and shedders. Here, a literature review is conducted to summarize the published findings in the virus genomic biology, transmission, epidemiology, pathogenesis, and diagnosis, which would shed light on acceleration of development of anti‐APPV strategies.     

Novel missense mutation in VPS33B is associated with isolated low gamma‐glutamyltransferase cholestasis: Attenuated,incomplete phenotype of arthrogryposis,renal dysfunction,and cholestasis syndrome

The typical phenotype of arthrogryposis, renal dysfunction, and cholestasis (ARC) syndrome involves three cardinal symptoms as the name describes, harboring biallelic mutations on VPS33B or VIPAS39. Except for ARC syndrome, low gamma‐glutamyltransferase (GGT) cholestasis often implies hereditary hepatopathy of different severity; however, some remain undiagnosed. Several monogenic defects typically with multiorgan manifestations may only present liver dysfunction at times, such as DGUOK defect and AGL defect. Previously, four VPS33B mutated cases were reported without arthrogryposis, or with less severe symptoms and longer lifespan, indicating the possibility of incomplete ARC phenotype of isolated hepatopathy. So we retrospectively reviewed all patients with confirmed VPS33B/VIPARS39 defect in our center and identified three presenting isolated low‐GGT cholestasis with intractable pruritus. Distinguished from others with typical ARC phenotype, these patients did not suffer the other two typical characteristics, survived much longer, and shared a novel missense VPS33B variation c.1726T>C, p.Cys576Arg, causing declined protein expression and abolished interaction with VIPAS39 in‐vitro. Serum bile acid profiles of our VPS33B/VIPAS39 mutated patients revealed similar changes to primary defect of bile salt export pump, among which those with isolated cholestasis phenotype had a higher level of total secondary bile acids than that with typical ARC phenotype, indicating the partial residual function of VPS33B.     

Functional characterization of CEP250 variant identified in nonsyndromic retinitis pigmentosa

Retinitis pigmentosa (RP) is the most common manifestation of inherited retinal diseases with high degree of genetic, allelic, and phenotypic heterogeneity. CEP250 encodes the C‐Nap1 protein and has been associated with various retinal phenotypes. Here, we report the identification of a mutation (c.562C>T, p.R188*) in the CEP250 in a consanguineous family with nonsyndromic RP. To gain insights into the molecular pathomechanism underlying CEP250 defects and the functional relevance of CEP250 variants in humans, we conducted a functional characterization of CEP250 variant using a novel Cep250 knockin mouse line. Remarkably, the disruption of Cep250 resulted in severe impairment of retinal function and significant retinal morphological alterations. The homozygous knockin mice showed significantly reduced retinal thickness and ERG responses. This study not only broadens the spectrum of phenotypes associated with CEP250 mutations, but also, for the first time, elucidates the function of CEP250 in photoreceptors using a newly established animal model.