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Introduction: Blau syndrome (BS) is a rare autoimmune disease. We report here an atypical case of BS. Case Presentation: We present a case of late-onset eye manifestations in a Chinese girl of 18 years old with sporadic BS, presenting with panuveitis. We performed comprehensive ocular examinations including fluorescein fundus angiography and indocyanine green angiography for her. The oral hormone plus local anti-inflammatory eye drops have well controlled the inflammation of her eyes. Conclusion: Our case highlights the necessity of systemic medical history inquiry for every eye discomfort. [ABSTRACT FROM AUTHOR]

A notable behavioural feature of X-linked retinoschisis (XLRS) is extensive structural schisis (splitting) of the outer plexiform and inner nuclear layers of the neurosensory retina, which is partly combined with complications related to vitreous hemorrhage, macular holes and retinal detachment. The present study aimed to identify the pathogenic gene mutation in a three-generation Chinese family with XLRS by whole-exome sequencing (WES). The clinical information of a three-generation Chinese family with cases of XLRS was collected. WES was performed for the proband. A comparison with the human reference genome sequence (hg38) and bioinformatic analysis were performed to reveal putative variants and Sanger sequencing was applied to verify mutations in this family and healthy control participants. Intraretinal cystic spaces were detected by optical coherence tomography imaging. Structures of the wild-type and mutant retinoschisin 1 (RS1) protein were modelled by PyMol. Almost all patients had a history of vision loss and abnormal blue-purple colour vision; however, the phenotypes of the 4 patients were distinctly different. There was no linear correlation between phenotypic severity and age. A recurrent RS1 (Xp22.2) mutation (NM_000330: c.559C>T) was detected, resulting in the p.Q187X variant. According to the protein model, this variant is likely pathogenic. The present study was the first to report that RS1:c.559C>T induces XLRS in a three-generation Chinese pedigree, with the mutation leading to premature termination of translation of the RS1 protein. WES was able to diagnose XLRS, which has the characteristics of clinical and genetic heterogeneity. [ABSTRACT FROM AUTHOR]

Congenital cataracts is the most common cause of visual impairment and blindness in children. Although there have been extensive studies into the pathogenesis of congenital cataracts, the pathogenic mechanism underlying the recurrent variant CRYBB2:c.62T>A(p.I21N) has not been previously reported. Thus, the present study aimed to use whole-exome sequencing (WES) to identify potential genetic variants and investigate how they may have induced the occurrence of cataracts in a four-generation Chinese family with congenital nuclear cataracts. The medical history of this family was recorded and WES was conducted for one proband. Sanger sequencing was used to verify the presence of the putative variant in all participants. PolyPhen-2, SIFT and ProtScale were used to analyze the effect of the identified variants on protein function and hydrophobicity, and Pymol was used to show the structure of the wild-type (Wt) and mutant β-crystallin B2 (CRYBB2) protein. Full-length Wt-CRYBB2 or mutant-CRYBB2 (I21N-CRYBB2) were fused to green fluorescent protein (GFP), and the recombinant plasmids were transfected into HeLa cells. Reverse transcription-quantitative PCR and western blotting were used to detect the expression levels of CRYBB2 mRNA and protein. Immunofluorescence and flow cytometry analyses were used to detect protein localization and apoptosis, respectively. A recurrent variant CRYBB2:c.62T>A(p.I21N) was identified in a four-generation Chinese family with congenital nuclear cataracts. Multiple-sequence alignment of CRYBB2 demonstrated that codon 21 was highly conserved. Pymol revealed that the structure of the I21N-CRYBB2 protein was distinct from that of Wt-CRYBB2. PolyPhen-2 predicted that it had a variant provean score 1.0, suggesting it was 'probably damaging', and SIFT predicted it had a variant provean score of -5.113, indicating it was 'deleterious'. ProtScale indicated that the hydrophobicity of the mutation site was significantly reduced. The protein expression levels of the I21N-CRYBB2 were decreased compared with the Wt-CRYBB2. Immunofluorescence analysis revealed that the variant I21N-CRYBB2 protein tended to accumulate around the nucleus, and flow cytometry analysis indicated that it increased cell apoptosis. Furthermore, I21N-CRYBB2 induced the activation of the unfolded protein response (UPR). In conclusion, a pathogenic variant of CRYBB2:c.62T>A(p.I21N) was identified via WES in a four-generation Chinese family with congenital nuclear cataracts. Through biological analysis, it was found that the variant induced abnormal protein aggregation, activated the UPR and triggered excessive cell apoptosis, which may lead to the occurrence of congenital nuclear cataracts in this family. [ABSTRACT FROM AUTHOR]

Purpose. To identify likely pathogenic variants in three families with congenital cataracts via panel-based exome sequencing. Methods. A panel containing 153 genes associated with congenital cataracts was designed. Genes were selected through reference to databases including the Human Gene Mutation Database (HGMD), Online Mendelian Inheritance in Man (OMIM), Genetic Home Reference, and the latest peer-reviewed publications on the genetics of hereditary cataracts. Panel-based exome sequencing was performed with the Illumina HiSeq X-Ten platform, and then the identified variants were confirmed with Sanger sequencing and evaluated according to the American College of Medical Genetics and Genomics (ACMG) criteria. Results. Three likely pathogenic variants were found. A novel CRYBB2: c.230G > T p.G77V variant was identified in family A, a novel CRYBB2: c.230G > A p.G77D variant was identified in family B, and a novel CRYGD: c.475delG p.A159Pfs∗9 variant was identified in family C. Conclusion. Panel-based exome sequencing revealed three likely pathogenic variants in three unrelated Chinese families with congenital cataracts. These data expand the genetic spectrum associated with congenital cataracts. [ABSTRACT FROM AUTHOR]

Liquid crystalline polymers (LCPs), especially liquid crystalline elastomers (LCEs) can generate ultrahigh shape change amplitude but has lower mechanical strength. Although some attempts have been tried to improve the mechanical performance of LCE, there are still limitations including complicated fabrication and high actuation temperature. Here, a versatile method is reported to fabricate light‐driven actuator by covalently cross‐linking polyurethane (PU) into LCP networks (PULCN). This new scheme is distinct from the previous interpenetrating network strategy, the hydrogen bonds and covalent bonds are used in this study to improve the miscibility of non‐liquid‐crystalline PU and LCP materials and enhance the stability of the composite system. This material not only possesses the shape memory properties of PU but shows shape‐changing behavior of LCPs. With a shrinkage ratio of 20% at the phase transition temperature, the prepared materials reached a maximum mechanical strength of 20 MPa, higher than conventional LCP. Meanwhile, the resulting film shows diverse and programmable initial shapes by constructing crosslinking density gradient across the thickness of the film. By integration of PULCN with near‐infrared light‐responsive polydopamine, local and sequential light control is achieved. This study may provide a new route for the fabrication of programmable and mechanically robust light‐driven soft actuator. [ABSTRACT FROM AUTHOR]

The injury of vascular endothelial cells is a crucial factor in the development of diabetic retinopathy (DR). PDLIM1 (a member of the PDZ and LIM protein family) has been reported to exert an essential function in vascular diseases. This study aimed to elucidate the role of PDLIM1 on retinal vascular endothelial cells in DR. Immunofluorescence staining was used to localize the expression of PDLIM1 in the mouse retina. In some tumor diseases, PDLIM1 has been reported to play a key role in regulating the Wnt pathway. However, no in-depth reports have been found in DR. Retinal capillary endothelial cells (RCECs) were treated with high-glucose and high-lipid (HG/HL) culture medium, and siRNA transfection to investigate the role of PDLIM1 in DR. PDLIM1 and Wnt3a expression was confirmed by qRT-PCR and western blotting. Flow cytometry, Transwell assay, and scratch assay were used to test the ability of cell apoptosis, migration, and invasion. PDLIM1 was mainly expressed in the retinal pigment epithelium (RPE), ganglion cell layer (GCL), inner plexus layer (IPL), and outer plexus layer (OPL). HG/HL increased Wnt3a levels and promoted cell's ability of apoptosis, migration, and invasion, which were reversed by the knockdown of PDLIM1. PDLIM1 was found to play a protective role in diabetic retinopathy by counter-regulating Wnt3a. PDLIM1 ameliorates cell apoptosis, migration, and invasion by negatively regulating Wnt3a in RCECs of DR, which suggests that PDLIM1 might be a promising therapeutic target for DR treatment. [ABSTRACT FROM AUTHOR]

The ability of some animals to rapidly change their colors can greatly improve their chances of escaping predators or hunting prey. A classic example is cephalopods, which can rapidly shift through a wide range of colors. This ability is based on the synergetic effect of the change of pigmentary and structural colors exhibited by their own two categories of color‐changing cells: supernatant chromatophores offer various pigmentary colors and lower iridophores or leucophores reflect the different structural colors by adjusting their periodicities. Here, a mechanochromic liquid crystalline elastomer with force‐induced synergetic pigmentary and structural color change, whose mechanosensitivity is enhanced by the stress‐concentration induced by the doped nanoparticle, is presented. The materials have a large color‐changing gamut and high mechanochromic sensitivity, which exhibit great potential in the field of mechanical detectors, sensors, and anti‐counterfeiting materials. [ABSTRACT FROM AUTHOR]

Background: Traditional Chinese medicine (TCM) has a long history of treating glaucoma with remarkable effects, but there is no clear conclusion on its mechanism. Methods: Network pharmacology and molecular docking were used to analyze the mechanism and targets of TCM in the treatment of glaucoma, and baicalein was used to treat chronic ocular hypertension animal models rats for observation. Results: The results of animal experiments showed that baicalein could significantly reduce intraocular pressure (IOP) in a rat model of chronic ocular hypertension and protect the structure of the retina and optic nerve, as shown by hematoxylin–eosin (H&E) staining and transmission electron microscopy (TEM). Reducing the apoptosis of retinal ganglion cells (RGCs) by upregulating the expression of the antiapoptotic protein BCL-2 is basically consistent with the results of molecular docking. In the network pharmacology analysis, many key proteins of biological pathways involved in the herbal therapeutic processes in glaucoma, such as threonine kinase 1 (AKT1, core protein of PI3K/AKT signaling), tumor protein p53 (TP53, a tumor suppressor gene coding tumor protein P53), signal transducer and activator of transcription 3 (STAT3, core protein of JAK/STAT signaling), interleukin 6 (IL-6) and interleukin 17 (IL-17, proinflammatory factors), were identified. Their interactions built complicated chain reactions in the process of glaucoma. Conclusion: By combining the analysis of network pharmacology and animal experimental results, baicalein could effectively improve the symptoms of glaucoma and reduce RGC apoptosis, suggesting that the potential mechanism of TCM in treating glaucoma is related to regulating inflammation and cellular immunity and reducing apoptosis. [ABSTRACT FROM AUTHOR]

Diabetic retinopathy (DR) is a leading cause of irreversible blindness globally. Qidengmingmu Capsule (QC) is a Chinese patent medicine used to treat DR, but the molecular mechanism of the treatment remains unknown. In this study, we identified and validated potential molecular mechanisms involved in the treatment of DR with QC via network pharmacology and molecular docking methods. The results of Ingredient-DR Target Network showed that 134 common targets and 20 active ingredients of QC were involved. According to the results of enrichment analysis, 2307 biological processes and 40 pathways were related to the treatment effects. Most of these processes and pathways were important for cell survival and were associated with many key factors in DR, such as vascular endothelial growth factor-A (VEGFA), hypoxia-inducible factor-1A (HIF-1Α), and tumor necrosis factor-α (TNFα). Based on the results of the PPI network and KEGG enrichment analyses, we selected AKT1, HIF-1α, VEGFA, TNFα and their corresponding active ingredients for molecular docking. According to the molecular docking results, several key targets of DR (including AKT1, HIF-1α, VEGFA, and TNFα) can form stable bonds with the corresponding active ingredients of QC. In conclusion, through network pharmacology methods, we found that potential biological mechanisms involved in the alleviation of DR by QC are related to multiple biological processes and signaling pathways. The molecular docking results also provide us with sound directions for further experiments. [ABSTRACT FROM AUTHOR]

Objective. Accumulating evidence suggests the critical role of autophagy in the pathogenesis of diabetic retinopathy (DR). In the current study, we aim to identify autophagy genes involved in DR via microarray analyses. Methods. Gene microarrays were performed to identify differentially expressed lncRNAs/mRNAs between normal and DR retinas. Gene Ontology and Kyoto Encyclopedia of Genes and Genomes analyses of lncRNA-coexpressed mRNAs were used to determine the related pathological pathways and biological modules. Real-time polymerase chain reactions (PCR) were conducted to validate the microarray analyses. Results. A total of 2474 significantly dysregulated lncRNAs and 959 differentially expressed mRNAs were identified in the retina of DR. Based upon Signalnet analysis, Bcl2, Gabarapl2, Atg4c, and Atg16L1 participated the process of cell death in DR. Moreover, real-time PCR revealed significant upregulation of Atg16L1. Conclusion. This study indicated the importance and potential role of Atg16L1, one of the autophagy genes, as a biomarker in DR development and progression. [ABSTRACT FROM AUTHOR]

• The liquid crystal phase temperature ranges of PDLC films are increased by using high clearing-point mesogenic compounds. • The working temperature range of the PDLC film is further expanded. • The effects of different mesogenic compound structures on the performance of PDLC films are studied. • The influence of mesogenic compound structure on the properties of the host liquid crystal is thoroughly investigated. As a type of smart dimming film, polymer dispersed liquid crystal (PDLC) film can meet the functional requirements in fields of displays and smart windows, but its narrow working temperature range is far from satisfactory for outdoor applications. To solve this problem, two types of high-clearing point mesogenic compounds with different end groups were introduced and the effects of their chemical structure on the working temperature range of PDLC film were investigated. Results show that the PDLC film doped with 10 wt% fluorine-containing LC molecule (3F) has a low driving voltage (<36 V) at −20 °C, but its contrast ratio (CR) value at the high temperature is disappointing. Furthermore, by adding 10 wt% cyano LC molecule (3 N), the film maintains an excellent CR value (62) at 90 °C. This work provides theoretical guidance and experimental support for broadening the working temperature of PDLC films, which is an important factor for their applications in automobile smart windows. [ABSTRACT FROM AUTHOR]

Purpose: To describe a manual prechop technique for splitting the nucleus of the lens using a recently developed reverse chopper. Methods: During the process, the reverse chopper and the Nagahara chopper are placed diagonally in the peripheral area of the nucleus of the lens after capsulorhexis. The reverse chopper and the Nagahara chopper then are pushed horizontally toward each other so they meet at the center of the lens to split the nucleus of lens into 2 parts. Results: In all cases, the reverse chopper was effective during the prechop procedure for hard nuclei, the nucleus of the lens remained in situ during the chopping process, and the reverse chopper did not retract the suspensory ligament in patients in whom the ligament was fragile. During the prechop procedure, no capsule breakage occurred, and the time and energy required for effective phacoemulsification were reduced significantly. Conclusions: The prechop technique using the reverse chopper can be applied for cases with grade III–V nuclei, overripe nuclei, and fragile suspensory ligaments. The procedure is simple, and the learning curve is not steep. [ABSTRACT FROM AUTHOR]

The structures of the liquid crystal (LC) molecules have a key role in impacting the electro-optical performance of a polymer dispersed liquid crystal (PDLC) film. In this paper, the relationship between the LC molecular structures and the electro-optical properties of PDLC films is investigated based on an unexplored cyano-terminated tolane compounds (CTTCs) doped E8 LCs/UV polymers system. Due to the high polarity of CTTCs, LCs doped with the cyano-terminated tolane (CTT) molecules exhibit high birefringence and large positive dielectric anisotropy. On the whole, PDLC films doped with the CTT molecules exhibit a lower driving voltage than that doped with the pure E8. More excitingly, PDLC films based on CTT molecules with larger length-to-width ratio and longer conjugated system show higher contrast ratio (CR) and faster response time. Eventually, the mechanism of the effects of CTT-based molecular structures and the relationship between the electro-optical performance of PDLC films and CTT molecules are illustrated. This work paves a new way for optimising the electro-optical properties of PDLC films. [ABSTRACT FROM AUTHOR]

Uveal melanoma (UM) is the most common primary intraocular tumor in adults, which has high frequency of metastasis to the liver, typically causing a fatal outcome. Chemo-resistance remains a major obstacle in the therapeutic approach to UM, leaving limited choice for treating UM. Other possible treatments have been explored but the results are yet to be evident. To improve therapy for UM, transcriptional suicide genes were transfected into the OCM-1 cell line. In the current study, OCM-1 cells transfected with lentiviral-meditated EGFP, cytosine deaminase (CD)/EGFP, and VP22-CD/EGFP were established. Of the three groups, we examined the cell growth in vitro and in vivo by using the MTT method with cell culture media and MRI in murine UM models. According to our results, the cell proliferation in the transfected CD/EGFP group was slower than the non-suicide gene group. The VP22-CD/EGFP group manifested superior cell cytotoxicity than the CD/EGFP group. Further analysis of MRI and fluorescent imaging of the murine UM model identified significant differences in tumor volume among the three groups. Collectively, our study demonstrated that CD/5-FC is a potent therapeutic approach for UM. With the efficacy of VP22, suicide gene-induced cytotoxicity was superior to applying CD alone. Taken together, we concluded that novel therapy with the VP22-CD suicide gene may further contribute to treatment of UM. [ABSTRACT FROM AUTHOR]

To study polymer-dispersed liquid crystal (PDLC) films doped with indium tin oxide (ITO) nanoparticles (NPs), samples were prepared by ultraviolet-initiated polymerisation based on the thiol-acrylate system. Owing to the interaction between PDLC system and ITO NPs, the content and the size of ITO NPs are the main determinants to the microstructure which plays an essential role on the electro-optical and anti-infrared properties of the PDLC films. In the polymer matrix, a novel microstructure consisting of a dense surface, micron-sized meshes and submicron meshes is found to benefit the better performances of the low driven voltage (20.7 V), the relatively high contrast ratio (8.3) and the lowest transmittance(500-2500 nm) on average at about 3.55% with maximum of merely 7.6%. Thus, it lays a solid foundation for the further investigations on the microstructure and the performance of the PDLC films. Meanwhile, it is proved that the PDLC film, improved performance through doping ITO NPs, is promising to be a superior choice in the field of energy-saving. [ABSTRACT FROM AUTHOR]

Aim: This study aimed to compare the effects of toric intraocular lens (IOL) implantation with a capsular tension ring and toric IOL implantation only in patients with axial myopic astigmatism who had undergone cataract surgery.Methods: Of 34 patients with axial myopia, 16 patients who had received IOL and capsular tension ring (CTR) implantation were included in the combined group and 18 patients who received toric IOL implantation only were included in the simple group. Uncorrected visual acuity (UCVA) and best-corrected visual acuity (BCVA) were evaluated by measuring subjective refraction, residual astigmatism, and the toric IOL axis six months post-surgery.Results: At six months postoperatively, the UCVA for the combined and simple groups was 4.6 ± 0.1 and 4.5 ± 0.2, respectively, a statistically significant difference (t = 3.531, P<0.05). The toric IOL in all of the cases was located in the capsular sac, but there were more cases with IOL rotation (12 eyes) in the simple group than in the combined group (4 eyes). The rotation angles were 20°~30° (one eye), 10°~20° (four eyes), and <10° (seven eyes) compared with 2°~5° (four eyes). The residual astigmatism was -0.50 ± 0.25 D in the combined group, not a significant difference from the predicted residual astigmatism (-0.35 ± 0.13 D). There was a significant difference in the simple group (-1.25 ± 0.33 D) when the predicted residual astigmatism was compared (-0.37 ± 0.11 D) (t = -9.511, P < 0.01).Conclusions: In patients with axial myopic astigmatism, CTR can effectively increase the rotational stability of a toric IOL, achieving improvement in corneal astigmatism and visual acuity. [ABSTRACT FROM AUTHOR]

To study effects of the crosslinking agent/diluents/thiol on morphology of the polymer matrix and the electro-optical properties of polymer-dispersed liquid crystal (PDLC) films, samples were prepared by ultraviolet (UV)-initiated polymerisation. Due to the interaction between thiol-acrylate reaction and acrylate monomers polymerisation, the sample compositions were the foremost determinant to the microstructures which in turn played an essential role on the electro-optical properties of the PDLC films. With the increasing content of the crosslinking agent, the LC droplet size decreased, while the thiol had a contrary effect on the LC droplet size. It was demonstrated that the superior properties of the low-driven voltage (37.2 V), the high contrast ratio (148.2), the short response time (14.9 ms) and the high saturation transmittance (86.6%) could benefit from a novel microstructure which had a dense surface and meshes with microspheres attached. It was of great significance for the optimisation and the potential applications of the PDLC films. [ABSTRACT FROM AUTHOR]

To improve the efficacy of anti-inflammatory factors in patients who undergo cataract surgery, poly(2-hydroxyethyl methacrylate-co-methyl methacrylate) (p(HEMA-co-MMA)) hydrogels containing β-cyclodextrin (β-CD) (pHEMA/MMA/β-CD) were designed and prepared as intraocular lens (IOLs) biomaterials that could be loaded with and achieve the sustained release of dexamethasone. A series of pHEMA/MMA/β-CD copolymers containing different ratios of β-CD (range, 2.77 to 10.24 wt.%) were obtained using thermal polymerization. The polymers had high transmittance at visible wavelengths and good biocompatibility with mouse connective tissue fibroblasts. Drug loading and release studies demonstrated that introducing β-CD into hydrogels increased loading efficiency and achieved the sustained release of the drug. Administering β-CD via hydrogels increased the equilibrium swelling ratio, elastic modulus and tensile strength. In addition, β-CD increased the hydrophilicity of the hydrogels, resulting in a lower water contact angle and higher cellular adhesion to the hydrogels. In summary, pHEMA/MMA/β-CD hydrogels show great potential as IOL biomaterials that are capable of maintaining the sustained release of anti-inflammatory drugs after cataract surgery. [ABSTRACT FROM AUTHOR]

The different fluorinated liquid crystal (LC) molecules doped to E8 were used as LC component to prepare polymer dispersed liquid crystal (PDLC) films. The mass fraction of the LC mixture is fixed 50.0 wt%. Results indicate that doping 8.0 wt% fluorinated LC molecule ME3CP to E8 significantly reduced the driving voltage of the PDLC films, and the driving voltage reduced with the rise of mass fraction of ME3CP. Besides, the terminal flexible chain length of the fluorinated LC molecule influenced the LC mixture properties based on E8, such as the dielectric anisotropy, birefringence and viscosity of the LC mixture, and the morphology and the electro-optical properties of PDLC films were controlled not only by the physical properties of the LC mixture, but also by the terminal flexible chain length of the fluorinated LC molecule . [ABSTRACT FROM AUTHOR]

As a smart dimming film, the polymer-dispersed liquid crystal film has potential application value in many fields. In this paper, an electrically controlled dimming film with a wide operating temperature range is investigated, which has fast response times and low driving voltage at −20 °C, and good shielding at 90 °C. The research can promote the application of the film in outdoor. [Display omitted] • The PDLC film has good electro-optical performances in the temperature range between −20 °C and 90 °C. • The working temperature of the PDLC film is broadened by using the liquid crystal with wide temperature range. • The effects of acrylate monomers and cross-linking agents on the working temperature of the PDLC film are investigated. • The influencing factors of the working temperature of the PDLC film are analyzed in detail. Polymer dispersed liquid crystal (PDLC) films have shown potential applications in various fields such as curtain walls, indoor partitions and smart windows due to their large-area and convenient manufacturing ability. However, the narrow working temperature range limits their outdoor applications, especially in automobiles. In this study, methods to improve the performances of PDLC films at high and low temperatures were explored. Liquid crystals (LCs) with wide temperature range were used and different polymerizable monomers and cross-linking agents matching the LCs were investigated. Results show that the prepared PDLC film has a better contrast ratio of 48 at a high temperature of 90 °C, and fast on-state and off-state response times of 74 ms and 895 ms at −20 °C, respectively, when using hexyl acrylate as monomer and 1,4-butanediol diacrylate as crosslinking agent. This research could promote the application of PDLC films to outdoor scenes like smart windows for automobiles. [ABSTRACT FROM AUTHOR]

To better perform space missions and develop human spaceflights, the eye health of astronauts is receiving increasing attention from researchers. In this study, we used prolonged tail suspension to simulate microgravity cephalad fluid shift in space to observe intraocular pressure (IOP) changes, retinal structure, and optic nerve damage in rats. We observed significant choroidal thickening and optic nerve demyelination lesions in the rats in each experimental group. At the cellular level, retinal ganglion cells (RGCs) survival was significantly reduced, optic nerve oligodendrocytes were reduced, and apoptotic factors and microglia-mediated inflammation-related factors were detected in both the retina and optic nerve. The severity of these changes increased with increasing tails suspension time. In conclusion, simulated long-term microgravity can lead to slight intraocular pressure fluctuations, choroidal thickening, reduced RGCs survival, and optic nerve demyelination in rats. • During the simulated long-term weightlessness, the IOP of rats fluctuated to some extent, but the fluctuation interval was within the range of normal IOP, the choroidal thickness of both eyes of rats was significantly thickened. • The expression of Iba1 and Cas3 in the retina and optic nerve was up-regulated during simulated long-term weightlessness. • The survival rate of retinal ganglion cells was significantly reduced. [ABSTRACT FROM AUTHOR]

A series of photo, temperature, and pH-responsive polymers have been synthesized by the quaternization of poly(dimethylaminoethyl methacrylate) (PDMAEMA) with 1-(bromomethyl)pyrene. Nanoparticles self-assembled from the pyrene-functionalized polymers in aqueous solution are demonstrated by transmission electron microscopy (TEM) and dynamic light scattering (DLS), the morphology of which can be changed under external stimulation by UV light, temperature, and pH. With the increase of the functionalization degree, the lower critical solution temperature (LCST) and the photo response of the pyrene-functionalized polymer increases, while the critical aggregation concentration (CAC) and the pH response decreases. The controlled release of encapsulated molecules such as Nile Red (NR)and anticancer drug doxorubicin (DOX) can be achieved under the triple stimulation from the self-assembled nanoparticles. [ABSTRACT FROM AUTHOR]

Polymer nanoparticles are prepared by self-assembly of visible light and pH sensitive perylene-functionalized copolymers which are synthesized by quaternization between 1-(bromomethyl)perylene and the dimethylaminoethyl units of poly(dimethylaminoethyl methacrylate) (PDMAEMA). The perylene-containing polymethacrylate segments afford the system visible light responsiveness and the unquaternized PDMAEMA segments afford the system pH responsiveness. The self-assembled nanoparticles exhibit a unique dual stimuli response. They can be photocleaved under visible light irradiation, shrunken to smaller nanoparticles at high pH, and swollen at low pH. The structural change endows the nanoparticle with great potential as a sensitive nanocarrier for controlled release of Nile Red and lysozyme under this stimulation. The visible light responsiveness and synergistic effect on the release of loaded molecules with the dual stimulation may obviate the need for harsh conditions such as UV light or extreme pH stimulation, rendering the system more applicable under mild conditions. [ABSTRACT FROM AUTHOR]

Purpose: To identify the genetic defect in a three-generation Chinese family with congenital cataracts. Methods: The phenotype of a three-generation Chinese family with congenital cataract was recruited. Detailed family history and clinical data of the family were recorded. Candidate genes sequencing was performed to screen out the disease-causing mutation. Bioinformatics analysis was performed to predict the function of mutant gene. Results: The phenotype of the family was identified as nuclear cataract. Direct sequencing revealed a c.161 G > T transversion in exon 1 of crystallin alpha-A (CRYAA). This mutation co-segregated with all affected individuals in the family and was not found in unaffected family members nor in the 100 unrelated controls. Bioinformatics analysis indicated that the 54th amino acid position was highly conserved and the mutation R54L caused an increase of local hydrophobicity around the substitution site. Conclusions: This study identified a novel disease-causing mutation c.161 G>T (p.R54L) in CRYAA in a Chinese family with autosomal dominant nuclear cataracts, this is the first report relating a G > T mutation in CRYAA leading to congenital nuclear cataract. [ABSTRACT FROM AUTHOR]

Congenital cataract is a clinically and genetically heterogeneous group of eye disorders that causes visual impairment and childhood blindness. The purpose of this study was to identify the genetic defect associated with autosomal dominant congenital perinuclear cataract in a Chinese family. A detailed family history and clinical data of the family were recorded, and candidate gene sequencing was performed to screen for mutation-causing disease in our study. Direct sequencing revealed a c.601G>A (p.E201K) transversion in exon 2 of GJA8. This mutation co-segregated with all affected individuals in the family and was not found in unaffected family members or 100 unrelated controls. The function and mechanism of novel GJA8 point mutation E201K in Chinese patients were then investigated in this study. We found E201K aberrantly located in cytoplasm and prevented its location in the plasma membrane. Induction of E201K expression led to a decrease in cell growth and viability by MTT (3-(4,5-Dimethylthiazol-2-yl)-2,5-diphenyltetrazolium bromide) assay. Our study provides important evidence that GJA8 is a disease-causing gene for congenital cataract and that mutation of GJA8 has a potential causative effect. [ABSTRACT FROM AUTHOR]

A series of novel transparent shape memory co-polymers, made from (meth)acrylate monomers, intended to be used as intraocular lens, was synthesized. The thermo-mechanical properties, shape memory properties and optical properties of the co-polymers could be adjusted by using monomers with various alkyl chain lengths. The cytocompatibility of the prepared co-polymers to L929 mouse connective tissue fibroblasts was demonstrated in vitro, and the co-polymers exhibited favorable cytocompatibility, supporting cell viability and proliferation. This study showed that the prepared co-polymers, which exhibited good flexibility, adjustable shape memory properties, high transparency and favorable cytocompatibility, could find great promising applications as intraocular lens. [ABSTRACT FROM AUTHOR]

Abstract: Combination chemotherapy has been a primary management for cancer. Thus a drug delivery system which can administer several drugs simultaneously and control the drug release at the cancer site is desired. Here we synthesized hyperbranched poly(amine-ester) (HPAE) macromers with different degrees of terminal Cation to make injectable hydrogels as a multi-drug delivery system. The aqueous solutions of the macromers were fast transformed into hydrogel at body temperature with a low concentration (0.05 wt%) of ammonium persulfate (APS) but no activator for accelerating the polymerization, since the HPAE macromer with tertiary amines and APS themselves formed a redox system as initiator. Three different types of drugs, doxorubicin hydrochloride (Dox), 5-fluorouracil (5FU) and leucovorin calcium (LC), were used as model drugs in this experiment. This system allows locally releasing single and/or combinations of anticancer drugs simultaneously by a controllable way. Behaviors of drug release can be controlled by the drug-loading methods or/and the Cation degree of macromers loaded with the drug molecules. The drug release period could be prolonged when the drug was loaded into the macromers with high content of CAE macromers exhibited good biocompatibility which was evaluated in L929 and MCF7 cell lines using MTT cell proliferation assay. The swelling behavior and degradation of HPAE hydrogels in vitro were also examined. These results suggest that the HPAE hydrogels hold great potential for use as injectable systems for locally delivering single and/or multiple drugs in chemotherapy of cancer. [Copyright &y& Elsevier]

Purpose: To identify the genetic defect in a four-generation Chinese family with autosomal dominant congenital nuclear cataract. Methods: Family history data were recorded. Clinical and ophthalmologic examinations were performed on family members. All the members were genotyped with microsatellite markers at loci associated with cataracts. Linkage analysis was performed after genotyping. Candidate genes were screened for mutation using direct sequencing. Results: Linkage analysis was obtained at markers D1S1653 (LOD score [ Z] = 1.50, recombination fraction [θ] = 0.0) and D1S498 (LOD score Z = 0.90, recombination fraction [θ] = 0.0), which encompasses the connexin 50 gene ( GJA8). Sequencing the coding regions of GJA8 revealed a novel, heterozygous c.773C>T transition that resulted in the substitution of a highly conserved serine by phenylalanine at codon 258 (S258F). Bioinformatics analysis showed that the mutation altered the hydrophobicity and secondary structure of the protein. This mutation co-segregated with the disease phenotype in all affected individuals and was not found in the unaffected family members or in 100 normal unrelated individuals. Conclusions: This study has identified a novel missense mutation located in the carboxyl terminus of GJA8 (S258F) associated with autosomal dominant nuclear cataract. [ABSTRACT FROM AUTHOR]

Abstract: The drawbacks of poloxamer hydrogel, such as dissolving quickly in aqueous solution, have limited its biomedical application. In order to improve the stability of hydrogel, a novel system was developed by combining the reversible thermo-sensitive property of poloxamer 407 and the thiol-ene reactivity between the acrylate and thiol groups. It was found that the sol–gel transition of the acrylate/thiol modified poloxamer 407 mixture could be achieved at body temperature even with a low concentration of 17.5 wt.%. Meanwhile, the reaction between the acrylate and thiol modified poloxamer 407s occurred spontaneously in mimic physiological conditions, thus the hydrogel with crosslinking structure was formed. As a result, the stability of the crosslinked hydrogel was enhanced remarkably, and the release time of the drug from the crosslinked hydrogel was about 4.0 times as long as that from the poloxamer 407 hydrogel. In vitro and in vivo experiments revealed that the biocompatibilities of the modified poloxamer 407 hydrogel were similar to that of poloxamer 407. These results indicate that the modified poloxamer 407s have potential applications in controlled drug release, tissue engineering and cell encapsulation etc. [Copyright &y& Elsevier]

Normal-mode polymer stabilised cholesteric liquid crystal (Ch-LC)/aerosil particles (APs) composites are prepared by photo-polymerisation of the photo-polymerisable monomer in the electrically induced homeotropically oriented cholesteric phase of a photo-polymerisable monomer/Ch-LC/APs mixture. On the application of an electric field, the composites can change from a light scattering state to a transparent state accompanied by the texture changing from a focal conic texture to an electrically induced homeotropic texture. Moreover, the process was greatly influenced by the addition of the APs. The focus of this study is on the effects of the APs on the electro-optical performance and the morphology of polymer network of the composites. The effect of the chiral dopant concentration on the electro-optical performance is also studied. [ABSTRACT FROM AUTHOR]

A photopolymerisable monomer/liquid crystal (LC)/chiral dopant/photoinitiator mixture with a smectic A (SmA*)-chiral nematic (N*) phase transition was prepared. After the SmA* phase was homeotropically oriented and then irradiated by ultraviolet light, a homeotropically oriented polymer network was formed in the SmA* phase and then a homeotropically oriented polymer network-stabilised liquid crystal (PSLC) film with a SmA*-N* phase transition was prepared. In the temperature range of the SmA* phase, the LC molecules were homeotropically oriented and the film exhibited a transparent state. However, in the temperature range of the N* phase, the film exhibited a strong light-scattering state owing to the fact that the LC molecules adopted a focal conic alignment affected by the homeotropically oriented polymer network. The strong light-scattering state of the N* phase could be changed into a transparent one when an electric field was applied. The focus of this study was on the effects of composition of the PSLC film on its thermo-optical and electro-optical properties. [ABSTRACT FROM AUTHOR]

Front Cover: Stimuli‐responsive nanoparticles that can respond to visible light and pH have been prepared from perylene‐functionalized poly(dimethylaminoethyl methacrylate). The controlled release of Nile Red and lysozyme from the nanoparticles stimulated by visible light and pH separately and synergistically is demonstrated. The polymer nanoparticles can be used as smart nanocarriers and could find applications in nanosciences and biotechnologies. Further information can be found in the article by J. Dong, R. Zhang, H. Wu, X. Zhan, H. Yang, S. Zhu, and G. Wang* on page 1255. [ABSTRACT FROM AUTHOR]