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Beryllium (Be) has been selected as the solid neutron multiplier material for a tritium breeding blanket module in ITER, which is also the primary option of the Chinese TBM program. But the irradiation swelling of beryllium is severe under high temperature, high irradiation damage and high doses of transmutation-induced helium. Advanced neutron multipliers with high stability at high temperature are desired for the demonstration power plant (DEMO) reactors and the China Fusion Engineering Test Reactor (CFETR). Beryllium alloys mainly composed of Be 12 M (M is W or Ti) phase were fabricated by HIP, which has a high melting point and high beryllium content. Beryllium and beryllide (Be 12 Ti and Be 12 W) samples were irradiated by helium ion with 30 keV and 1 × 10 18 cm -2 at RT. The microstructures of Be, Be 12 Ti and Be 12 W samples were analyzed by SEM and TEM before and after ion irradiation. The average size of the first blistering on the surface of Be-W alloy is about 0.8 μm, and that of secondary blistering is about 79 nm. The surface blistering rates of the beryllium and beryllide samples were also compared. These results may provide a preliminary experimental basis for evaluating the irradiation swelling resistance of beryllium alloy.

Background: The main purpose of this paper is to introduce a method that can accurately locate the posterior capsule of the lens to facilitate a relatively complete resection of the anterior vitreous body., Methods: A total of 51 patients in the experimental group and control group were enrolled in this study. Phacoemulsification combined with vitrectomy was performed in all cases. After the cataract procedure was completed in the control group, the surgeon performed a conventional anterior vitrectomy with the operative eye. In the experimental group, anterior vitrectomy was performed according to the threadiness corrugation of the posterior capsule of the lens. During the operation, with the help of triamcinolone, two surgeons confirmed the resection of the anterior vitreous cortex; the best corrected visual acuity and intraocular pressure of all patients were recorded at 1 week, 1 month and 3 months after surgery., Results: Fifty patients underwent phacoemulsification combined with vitrectomy, except one patient in the experimental group who was lost to follow-up. After surgery, no significant complications were observed in all patients except two patients in the control group with temporary increases in intraocular pressure. There was no significant difference in preoperative visual acuity between the two groups (t = 0.83, P = 0.25). Both groups had varying degrees of improvement in best corrected visual acuity at 1 week, 1 month and 3 months after surgery. Moreover, there was no significant difference in BCVA between the two groups at the three follow-up time points (t=-1.15, -1.65, -1.09, P = 0.53, 0.21, 0.23). After surgery, no significant complications were observed in all patients except two patients in the control group with temporary increases in intraocular pressure. Incomplete resection of the anterior vitreous cortex was observed in 2 patients in each group, but there was no significant difference (χ 2  = 7.81, P > 0.05)., Conclusion: In the process of cataract surgery combined with vitrectomy, thready corrugation appears in the posterior capsule of the lens and is an important sign of its localization. Anterior vitrectomy can be accomplished safely and effectively with the help of thread-like corrugation, and the surgical effect is almost the same as that of traditional surgery. Especially suitable for beginners in vitreous surgery., (© 2024. The Author(s).)

Recently, the investigation of the dielectric properties of glasses in the GHz frequency range has attracted great interest for use in printed circuit boards (PCBs) as a reinforcing material in the application of high-speed 5G/6G communications. In particular, glasses with low dielectric properties are a prerequisite for high-frequency applications. In this study, the GHz dielectric properties of alkali-free aluminoborosilicate glasses without and with La 2 O 3 were analyzed using the Clausius-Mossotti equation where both the electronic and ionic polarizabilities contribute to the dielectric constant. The dielectric polarizability (α D ) and oxide ion polarizability (α O2- ) were calculated from the measured dielectric constant (ε GHz ) at 1 GHz and the glass density. The dielectric constants (ε opt ) at the optical frequencies and electronic polarizabilities (α e ) of the glasses were calculated from the refractive index measured at 633 nm and the glass density. The ε GHz values were found to be significantly higher than the ε opt values in both series of glasses, due to the ionic polarizability (α i ), which contributes additionally to the ε GHz . The lower dielectric constants of the La 2 O 3 -incoporated glasses than that of the reference glass without La 2 O 3 may be due to the lower ionic polarizability originated from the incorporation of the high cation field strength of the La 3+ ions.

The leopard coral grouper ( Plectropomus leopardus ) is a species of significant economic importance. Although artificial cultivation of P. leopardus has thrived in recent decades, the advancement of selective breeding has been hindered by the lack of comprehensive population genomic data. In this study, we identified over 8.73 million single nucleotide polymorphisms (SNPs) through whole-genome resequencing of 326 individuals spanning six distinct groups. Furthermore, we categorized 226 individuals with high-coverage sequencing depth (≥14×) into eight clusters based on their genetic profiles and phylogenetic relationships. Notably, four of these clusters exhibited pronounced genetic differentiation compared with the other populations. To identify potentially advantageous loci for P. leopardus , we examined genomic regions exhibiting selective sweeps by analyzing the nucleotide diversity ( θπ ) and fixation index ( F ST ) in these four clusters. Using these high-coverage resequencing data, we successfully constructed the first haplotype reference panel specific to P. leopardus . This achievement holds promise for enabling high-quality, cost-effective imputation methods. Additionally, we combined low-coverage sequencing data with imputation techniques for a genome-wide association study, aiming to identify candidate SNP loci and genes associated with growth traits. A significant concentration of these genes was observed on chromosome 17, which is primarily involved in skeletal muscle and embryonic development and cell proliferation. Notably, our detailed investigation of growth-related SNPs across the eight clusters revealed that cluster 5 harbored the most promising candidate SNPs, showing potential for genetic selective breeding efforts. These findings provide a robust toolkit and valuable insights into the management of germplasm resources and genome-driven breeding initiatives targeting P. leopardus .

Following transected spinal cord injury (SCI), there is a critical need to restore nerve conduction at the injury site and activate the silent neural circuits caudal to the injury to promote the recovery of voluntary movement. In this study, we generated a rat model of SCI, constructed neural stem cell (NSC)-derived spinal cord-like tissue (SCLT), and evaluated its ability to replace injured spinal cord and repair nerve conduction in the spinal cord as a neuronal relay. The lumbosacral spinal cord was further activated with tail nerve electrical stimulation (TNES) as a synergistic electrical stimulation to better receive the neural information transmitted by the SCLT. Next, we investigated the neuromodulatory mechanism underlying the action of TNES and its synergism with SCLT in SCI repair. TNES promoted the regeneration and remyelination of axons and increased the proportion of glutamatergic neurons in SCLT to transmit brain-derived neural information more efficiently to the caudal spinal cord. TNES also increased the innervation of motor neurons to hindlimb muscle and improved the microenvironment of muscle tissue, resulting in effective prevention of hindlimb muscle atrophy and enhanced muscle mitochondrial energy metabolism. Tracing of the neural circuits of the sciatic nerve and tail nerve identified the mechanisms responsible for the synergistic effects of SCLT transplantation and TNES in activating central pattern generator (CPG) neural circuits and promoting voluntary motor function recovery in rats. The combination of SCLT and TNES is expected to provide a new breakthrough for patients with SCI to restore voluntary movement and control their muscles., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2023 Elsevier Ltd. All rights reserved.)

The treatment of refractory Glaucoma is a difficult problem in clinical ophthalmology. For refractory glaucoma patients with hyphema, shallow anterior chamber, anterior conglutination of peripheral chamber angle, corneal endothelium dystrophy or decompensated, at present, there is no effective treatment. In order to solve this problem, a new type posterior integral glaucoma valve with IOP control device was designed using medical titanium alloy, and the valve model was established by Abaqus software, and the stiffness and preload of the valve were analyzed by finite element method. The results showed that the opening and closing of the valve were controlled automatically by the pressure difference between the front and back of the valve, and the opening and flow rate of the valve increase dynamically with the increase of intraocular pressure, and finally reached the set ideal IOP value of steady state.

The inability of adult mammals to recover function lost after severe spinal cord injury (SCI) has been known for millennia and is mainly attributed to a failure of brain-derived nerve fiber regeneration across the lesion. Potential approaches to re-establishing locomotor function rely on neuronal relays to reconnect the segregated neural networks of the spinal cord. Intense research over the past 30 years has focused on endogenous and exogenous neuronal relays, but progress has been slow and the results often controversial. Treatments with stem cell-derived neuronal relays alone or together with functional electrical stimulation offer the possibility of improved repair of neuronal networks. In this review, we focus on approaches to recovery of motor function in paralyzed patients after severe SCI based on novel therapies such as implantation of stem cell-derived neuronal relays and functional electrical stimulation. Recent research progress offers hope that SCI patients will one day be able to recover motor function and sensory perception., (Copyright © 2021 The Authors. Published by Elsevier Ltd.. All rights reserved.)

Axon regeneration and remyelination of the damaged region is the most common repair strategy for spinal cord injury. However, achieving good outcome remains difficult. Our previous study showed that porcine decellularized optic nerve better mimics the extracellular matrix of the embryonic porcine optic nerve and promotes the directional growth of dorsal root ganglion neurites. However, it has not been reported whether this material promotes axonal regeneration in vivo. In the present study, a porcine decellularized optic nerve was seeded with neurotrophin-3-overexpressing Schwann cells. This functional scaffold promoted the directional growth and remyelination of regenerating axons. In vitro, the porcine decellularized optic nerve contained many straight, longitudinal channels with a uniform distribution, and microscopic pores were present in the channel wall. The spatial micro topological structure and extracellular matrix were conducive to the adhesion, survival and migration of neural stem cells. The scaffold promoted the directional growth of dorsal root ganglion neurites, and showed strong potential for myelin regeneration. Furthermore, we transplanted the porcine decellularized optic nerve containing neurotrophin-3-overexpressing Schwann cells in a rat model of T10 spinal cord defect in vivo. Four weeks later, the regenerating axons grew straight, the myelin sheath in the injured/transplanted area recovered its structure, and simultaneously, the number of inflammatory cells and the expression of chondroitin sulfate proteoglycans were reduced. Together, these findings suggest that porcine decellularized optic nerve loaded with Schwann cells overexpressing neurotrophin-3 promotes the directional growth of regenerating spinal cord axons as well as myelin regeneration. All procedures involving animals were conducted in accordance with the ethical standards of the Institutional Animal Care and Use Committee of Sun Yat-sen University (approval No. SYSU-IACUC-2019-B034) on February 28, 2019., Competing Interests: None

Directional axon regeneration and remyelination are crucial for repair of spinal cord injury (SCI), but existing treatments do not effectively promote those processes. Here, we propose a strategy for construction of niche-specific spinal white matter-like tissue (WMLT) using decellularized optic nerve (DON) loaded with neurotrophin-3 (NT-3)-overexpressing oligodendrocyte precursor cells. A rat model with a white matter defect in the dorsal spinal cord of the T10 segment was used. The WMLT transplantation group showed significant improvement in coordinated motor functions compared with the control groups. WMLT transplants integrated well with host spinal cord white matter, effectively addressing several barriers to directional axonal regeneration and myelination during SCI repair. In WMLT, laminin was found to promote development of oligodendroglial lineage (OL) cells by binding to laminin receptors. Interestingly, laminin could also guide linear axon regeneration via interactions with specific integrins on the axon surface. The WMLT developed here utilizes the unique microstructure and bioactive matrix of DON to create a niche rich in laminin, NT-3 and OL cells to achieve significant structural repair of SCI. Our protocol can help to promote research on repair of nerve injury and construction of neural tissues and organoids that form specific cell niches., Competing Interests: The authors declare no conflict of interest., (© 2021 The Authors.)

Biofabrication with various hydrogel systems allows the production of tissue or organ constructs in vitro to address various challenges in healthcare and medicine. In particular, photocrosslinkable hydrogels have great advantages such as excellent spatial and temporal selectivity and low processing cost and energy requirements. However, inefficient polymerization kinetics of commercialized photoinitiators upon exposure to UV-A radiation or visible light increase processing time, often compromising cell viability. In this study, we developed a hydrogel crosslinking system which exhibited efficient crosslinking properties and desired mechanical properties with high cell viability, through a dual-photoinitiator approach. Through the co-existence of Irgacure 2959 and VA-086, the overall crosslinking process was completed with a minimal UV dosage during a significantly reduced crosslinking time, producing mechanically robust hydrogel constructs, while most encapsulated cells within the hydrogel constructs remained viable. Moreover, we fabricated a large PEGDA hydrogel construct with a single microchannel as a proof of concept for hydrogels with vasculature to demonstrate the versatility of the system. Our dual-photoinitiator approach allowed the production of this photocrosslinkable hydrogel system with microchannels, significantly improving cell viability and processing efficiency, yet maintaining good mechanical stability. Taken together, we envision the concurrent use of photoinitiators, Irgacure 2959 and VA-086, opening potential avenues for the utilization of various photocrosslinkable hydrogel systems in perfusable large artificial tissue for in vivo and ex vivo applications with improved processing efficiency and cell viability.

Objective: To observe the efficacy and feasibility of a new therapy using a combination of different anticholinergic eyedrops in controlling myopia progression and axial prolongation in adolescents. Methods: Between July 2013 and June 2014, a total of 150 myopia adolescents aged 6-12 years were recruited at the clinic of Tongji Hospital in Shanghai. Participants were assigned in a 1∶2∶2 ratio to placebo group (no medication), combined treatment group (0.5% racanisodamine eyedrops were used twice a day during semesters, 1% atropine eyedrops were used before sleep during vacation) and atropine group (1% atropine eyedrops were used before sleep everyday). All subjects wore glasses. Visual acuity, best corrected visual acuity, cycloplegic refraction, corneal curvature, axial length, intraocular pressure, fundus and adverse events were recorded every 6 months during follow-up for 24 months. Results: At baseline, there was no significant difference in age,equivalent spherical mirror number and axial length among the three groups (all P> 0.05). At the end of the second year,the mean myopia progression (changes of spherical equivalent) was -2.34 (-2.93,-1.75) D,-0.63 (-1.00,-0.50) D and -0.25 (-0.50,-0.06) D in placebo group, combined treatment group and atropine group, respectively ( P< 0.001), and there was statistically significant difference between each two groups (all P< 0.001). The axial length change of each group were (1.51±0.23) mm, (0.69±0.30) mm and (0.31±0.30) mm, respectively ( P< 0.001), and there was statistically significant difference between each two groups (all P< 0.001). Conclusion: Therapy using a combination of different anticholinergic eyedrops can effectively control the progression of myopia and axial prolongation in adolescents, and increase the compliance of children and the safety of drug use.

The effect of γ-ray irradiation on the surface plasmon resonance (SPR) sensing capability of refractive index (n = 1.418⁻1.448) of the silica glass optical fiber comprised of germano-silicate glass cladding embedded with Au nano-particles (NPs) was investigated. As the γ-ray irradiation increased from 1 h to 3 h with the dose rate of 1190 Gy/h, the morphology of the Au NPs and the SPR spectrum were found to change. The average diameter of Au NPs increased with the aspect ratio from 1 to 2, and the nano-particles became grown to the clusters. The SPR band wavelength shifted towards a longer wavelength with the increase of total dose of γ-ray irradiation regardless of the corresponding refractive indices. The SPR sensitivities (wavelength/refractive index unit, nm/RIU) also increased from 407 nm/RIU to 3553 nm/RIU, 1483 nm/RIU, and 2335 nm/RIU after the γ-ray irradiation at a total dose of 1190 Gy, 2380 Gy, and 3570 Gy, respectively.

Biofabrication technologies have endowed us with the capability to fabricate complex biological constructs. However, cytotoxic biofabrication conditions have been a major challenge for their clinical application, leading to a trade-off between cell viability and scalability of biofabricated constructs. Taking inspiration from nature, we proposed a cell protection strategy which mimicks the protected and dormant state of plant seeds in adverse external conditions and their germination in response to appropriate environmental cues. Applying this bioinspired strategy to biofabrication, we successfully preserved cell viability and enhanced the seeding of cell-laden biofabricated constructs via a cytoprotective pyrogallol (PG)-alginate encapsulation system. Our cytoprotective encapsulation technology utilizes PG-triggered sporulation and germination processes to preserve cells, is mechanically robust, chemically resistant, and highly customizable to adequately match cell protectability with cytotoxicity of biofabrication conditions. More importantly, the facile and tunable decapsulation of our PG-alginate system allows for effective germination of dormant cells, under typical culture conditions. With this approach, we have successfully achieved a biofabrication process which is reproducible, scalable, and provided a practical solution for off-the-shelf availability, shipping and temporary storage of fabricated bio-constructs.

Kelp are main iodine accumulators in the ocean, and their growth and photosynthesis are likely to benefit from elevated seawater CO 2 levels due to ocean acidification. However, there are currently no data on the effects of ocean acidification on iodine metabolism in kelp. As key primary producers in coastal ecosystems worldwide, any change in their iodine metabolism caused by climate change will potentially have important consequences for global geochemical cycles of iodine, including iodine levels of coastal food webs that underpin the nutrition of billions of humans around the world. Here, we found that elevated pCO 2 enhanced growth and increased iodine accumulation not only in the model kelp Saccharina japonica using both short-term laboratory experiment and long-term in situ mesocosms, but also in several other edible and ecologically significant seaweeds using long-term in situ mesocosms. Transcriptomic and proteomic analysis of S. japonica revealed that most vanadium-dependent haloperoxidase genes involved in iodine efflux during oxidative stress are down-regulated under increasing pCO 2 , suggesting that ocean acidification alleviates oxidative stress in kelp, which might contribute to their enhanced growth. When consumed by abalone (Haliotis discus), elevated iodine concentrations in S. japonica caused increased iodine accumulation in abalone, accompanied by reduced synthesis of thyroid hormones. Thus, our results suggest that kelp will benefit from ocean acidification by a reduction in environmental stress however; iodine levels, in kelp-based coastal food webs will increase, with potential impacts on biogeochemical cycles of iodine in coastal ecosystems., (© 2018 John Wiley & Sons Ltd.)

All-optical fiber magnetic field sensor based on the Gd₂O₃ nano-particles (NPs)-doped alumino-silicate glass optical fiber was developed, and its temperature and vibration dependence on the Faraday Effect were investigated. Uniformly embedded Gd₂O₃ NPs were identified to form in the core of the fiber, and the measured absorption peaks of the fiber appearing at 377 nm, 443 nm, and 551 nm were attributed to the Gd₂O₃ NPs incorporated in the fiber core. The Faraday rotation angle (FRA) of the linearly polarized light was measured at 650 nm with the induced magnetic field by the solenoid. The Faraday rotation angle was found to increase linearly with the magnetic field, and it was about 18.16° ± 0.048° at 0.142 Tesla (T) at temperatures of 25 °C-120 °C, by which the estimated Verdet constant was 3.19 rad/(T∙m) ± 0.01 rad/(T∙m). The variation of the FRA with time at 0.142 T and 120 °C was negligibly small (-9.78 × 10 -4 °/min). The variation of the FRA under the mechanical vibration with the acceleration below 10 g and the frequency above 50 Hz was within 0.5°., Competing Interests: The authors declare no conflict of interest.

Alumino-germano-silicate glass optical fiber incorporated with Gd2O3 nano-particles (NPs) was developed by using the modified chemical vapor deposition and the drawing process. The formation of spherical Gd2O3 NPs in the fiber core with average diameter of 10.8 nm was confirmed by the TEM. The distinct absorption peaks in the fiber preform appearing in the UV region at 205, 247, 253, 274, and 312 nm were due to the incorporated Gd2O3 NPs via reorganization of the seven 4f electrons into various multiplets of Gd ions. In the case of the optical fiber obtained by drawing of the preform at high temperature about 2150 °C, absorption peaks due to Gd2O3 NPs were found to appear at 383 and 455 nm, which were red-shifted from 274 and 312 nm of the preform, respectively, and it may be due to increase in the size of Gd2O3 NPs after the drawing process. To investigate the photoluminescence (PL) property for UV sensor applications, the PL of the fiber was obtained by illumination of the Xenon-lamp. A PL band appeared in the wavelength band from 370 nm to 450 nm, centering at about 400 nm, which can be attributed to the presence of Gd2O3 NPs embedded in the fiber core. It was also found that the PL intensity at 400 nm showed linear dependence with the excitation power from 0 to 400 W.

Three-dimensional (3D) printing of hydrogels is now an attractive area of research due to its capability to fabricate intricate, complex and highly customizable scaffold structures that can support cell adhesion and promote cell infiltration for tissue engineering. However, pure hydrogels alone lack the necessary mechanical stability and are too easily degraded to be used as printing ink. To overcome this problem, significant progress has been made in the 3D printing of hydrogel composites with improved mechanical performance and biofunctionality. Herein, we provide a brief overview of existing hydrogel composite 3D printing techniques including laser based-3D printing, nozzle based-3D printing, and inkjet printer based-3D printing systems. Based on the type of additives, we will discuss four main hydrogel composite systems in this review: polymer- or hydrogel-hydrogel composites, particle-reinforced hydrogel composites, fiber-reinforced hydrogel composites, and anisotropic filler-reinforced hydrogel composites. Additionally, several emerging potential applications of hydrogel composites in the field of tissue engineering and their accompanying challenges are discussed in parallel., Competing Interests: No conflict of interest was reported by all authors. This research was supported by AcRF Tier 1 grant 2017- T1-001-246 (RG51/17) from Ministry of Education of Singapore, and Basic Science Research Program (No. 2015R1D1A1A01057311 & 2017R1A6A3A03008914) through the National Research Foundation of Korea (NRF) funded by the Ministry of Science, ICT & Future Planning., (Copyright: © 2018 Jang T-S, et al.)

The aim of this study was to determine the association between two SNPs (rs2235371 and rs2013162) in the interferon regulatory factor 6 (IRF6) gene and non-syndromic cleft palate (NSCP) in northeast China. We genotyped these two SNPs in 104 NSCP cases, as well as in 178 parents and 300 controls. Case-control and case-parent analyses were performed using χ2 tests and family-based association tests (FBAT). Results indicated that there were significant differences in both genotypic and allelic distributions between patients and controls at rs2235371 and rs2013162 in the IRF6 gene. Case-parent analysis revealed over-transmission of the C allele in rs2235371 and the A allele in rs2013162. Lastly, FBAT showed over-transmission of the CA haplotype. This study demonstrated that the two SNPs, rs2235371 and rs2013162, are strongly associated with NSCP in the northeast Chinese population.

A novel surface plasmon resonance (SPR) sensor based on specialty optical fiber having its cladding doped with Au nano-particles (NPs) was developed by modified chemical vapor deposition process. To optimize the SPR absorption and sensitivity of the fiber SPR sensor, effect of the fiber length (20 cm-90 cm) on sensing capability of refractive index (n = 1.418-1.448) was investigated. Absorption peaks appearing at 392 and 790 nm were due to SPR from Au NPs in the cladding region of the optical fiber. The SPR was found to occur at particular wavelengths around 390 nm for the corresponding refractive indices regardless of the length of the fiber, increased with the increase of the index. The measured SPR sensitivities (wavelength/RIU) of the fiber were estimated to be 407 nm/RIU, 217 nm/RIU, and 54 nm/RIU with the fiber lengths of 20 cm, 45 cm, and 90 cm, respectively. The SPR absorption intensity and FWHM decreased with the increase of the fiber length because the propagation loss of the signal through the fiber cladding region increased.

The germano-silicate optical fiber incorporated with Ge nanoparticles with enhanced optical nonlinearity was developed by using modified chemical vapor deposition and drawing processes. A broad photoluminescence band obtained by pumping with the 404 nm superluminescent diode was found to appear from 540 nm to 1,000 nm. The non-resonant nonlinear refractive index, n2, of the fiber measured by the continuous wave self-phase modulation method was 4.95 x 10(-20) m2/W due to the incorporated Ge nanoparticles in the fiber core. The enhancement of the non-resonant optical non-linearity may be due to the creation of the NBOs and other defects from the incorporated Ge-NPs in the fiber core.

We have investigated gamma-ray radiation response at 1550 nm of fluorine-doped radiation hard single-mode optical fiber. Radiation-induced attenuation (RIA) of the optical fiber was measured under intermittent gamma-ray irradiations with dose rate of ~10 kGy/h. No radiation hardening effect on the RIA by the gamma-ray pre-dose was found when the exposed fiber was bleached for long periods of time (27~47 days) at room-temperature. Photo-bleaching scheme upon 980 nm LD pumping has proven to be an effective deterrent to the RIA, particularly by suppressing the incipient RIA due to room-temperature unstable self-trapped hole defects (STHs). Large temperature dependence of the RIA of the optical fiber together with the photo-bleaching effect are worthy of note for reinforcing its radiation hard characteristics.

We studied four Chinese families with pure hereditary spastic paraplegia (HSP) to investigate the clinical features and associated genetic mutations. Linkage analysis was performed for all families to map the disease locus onto autosomal chromosomes, and related loci involved in HSP on the X chromosome were also examined. Polymerase chain reaction (PCR) sequencing was used to detect gene mutations. To confirm the influence of a splice-site mutation on mRNA, we used reverse transcription-PCR and direct sequencing. Linkage analysis and ATL1 gene sequencing of amniocytes were performed for prenatal genetic diagnosis. One missense variant (c.1517T>A) and a splice-site mutation (c.1245+1G>A) in SPAST, and two missense variants (c.715C>T, c.1204T>G) in ATL1 were identified. The c.1245+1G>A mutation caused a deletion of exon 9 in the SPAST gene. Prenatal genetic diagnosis showed that fetus did not carry the ALT1 c.1204T>G mutation. Follow-up was maintained for 5 years, and the negative result was confirmed by evidence of a healthy growing boy. We identified two novel mutations and two previously reported mutations in SPAST and ATL1, respectively. The family with the ATL1 c.1204T>G mutation exhibited male-lethality, female infancy-onset, and pseudo- X-linked dominant transmission, which had never been previously reported for HSP. Characteristic facial features were also noticed. The boy on whom prenatal gene diagnosis was performed is healthy and without unusual facies, suggesting that the c.1204T>G mutation might be related to these features. The results extend the genetic spectrum of HSP and suggest that linkage analysis remains a powerful tool in gene discovery studies.

Non-syndromic cleft lip with or without cleft palate (NSCL/P) is a common birth defect. Several WNT genes are involved in craniofacial embryogenesis, and therefore may play an important role in the etiology of NSCL/P. Two SNPs (rs3809857 and rs9890413) in the WNT3 gene were subjected to case-control and case-parent analysis by polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP) in 236 unrelated patients with NSCL/P, including 128 elementary families (185 mothers and 154 fathers), and 400 control individuals from northeast China. The rs3809857 SNP, under the assumption of a dominant model, was found to induce a 2-fold lower risk of NSCL/P ORGG vs GT + TT = 0.605, 95%CI = 0.436-0.839, P = 0.003). Moreover, the family-based association test revealed an under-transmission for the minor allele T. On the other hand, we observed a significant association in the case-control and case-parent analysis of the SNP rs9890413. In addition, the P values for the haplotype of rs3809857-rs9890413 were observed to be statistically significant (P = 0.004). In conclusion, our study confirmed the association between the WNT3 variant and NSCL/P in the population tested.

We report a novel radial-firing optical fiber tip containing a conical-shaped air-pocket fabricated by deforming a hollow optical fiber using electric arc-discharge process. The hollow optical fiber was fusion spliced with a conventional optical fiber, simultaneously deforming into the intagliated conical-shaped region along the longitudinal fiber-axis of the fiber due to the gradual collapse of the cavity of the hollow optical fiber. Then the distal-end of the hollow optical fiber was sealed by the additional arc-discharge in order to obstruct the inflow of an external bio-substance or liquid to the inner air surface during the surgical operations, resulting in the formation of encased air-pocket in the silica glass fiber. Due to the total internal reflection of the laser beam at the conical-shaped air surface, the laser beam (λ = 632.8 nm) was deflected to the circumferential direction up to 87 degree with respect to the fiber-axis.

Congenital contractural arachnodactyly (CCA, OMIM: 121050) is an autosomal dominant condition that shares skeletal features with Marfan syndrome (MFS, OMIM: 154700), including contractures, arachnodactyly, dolichostenomelia, scoliosis, crumpled ears and pectus deformities but excluding the ocular and cardiovascular complications that characterize MFS. These two similar syndromes result from mutations in two genes belonging to the fibrillin family, FBN1 and FBN2, respectively. We successfully identified a novel FBN2 mutation (C1406R) in a Chinese family with CCA for over five generations. This mutation was detected in the patients of this family but not in the seven unaffected family members or 100 normal individuals. SIFT and PolyPhen analyses suggested that the mutation was pathogenic. We identified a missense mutation in the calcium binding-epidermal growth factor (cbEGF)-like domain. Our study extends the mutation spectrum of CCA and confirms a relationship between mutations in the FBN2 gene and the clinical findings of CCA.

A highly sensitive temperature sensor was made by use of a side-hole glass fiber filled with indium metal, and its optical properties were investigated. The temperature sensitivity of the fiber-optic temperature sensor was dλ/dT=-7.38 nm/K. The temperature sensitivity was also examined in sensors made by different lengths of the side-hole fiber and the indium-filled fiber region. The temperature sensitivity could be varied in the range of -1.83 to -7.38 nm/K by changing the relative length between the side-hole fiber and the indium-filled fiber region.

We have developed a cost-effective, high-capacity Er-doped fiber amplifier based on the high-power white LED and a large-core Er-doped fiber. Er ions of the fiber with absorption bands at 453, 488, 523, and 654 nm were simultaneously excited by LED pumping and amplification with optical gain over 12 dB at 1550 nm was obtained.

A fiber-optic interferometric probe based on a two-mode fiber (TMF) is proposed and demonstrated for measuring the thermo-optic coefficients (TOCs) of liquid samples. The proposed probe can be simply fabricated by fusion-splicing a short piece of TMF to a lead single mode fiber (SMF) with small lateral offset, which makes interference between LP(01) and LP(02) modes. The sensing responses of the probe to temperature and surrounding refractive index (SRI) have been experimentally investigated to show the capability of simultaneous measurements; the phase change of the reflection spectrum was related to temperature variation and the intensity change was to SRI variation. The data analysis is made not only in the spectral domain but in the Fourier domain also to effectively quantify the measurements. The TOCs of several liquid samples including water, ethanol, and acetone have been obtained with the proposed method.

The effect of the infiltration pressure on the birefringent properties of a side-hole fiber filled with indium was investigated by the fiber-optic Sagnac loop interferometry. The fiber was made at the various gas pressures during the metal infiltration process. It was found that the birefringence of the fiber strongly decreased from 5.55×10(-4) to 1.68×10(-4) with the increase of the pressure from 15 to 45 bars, due to the compensation effect of the pressure applied during the infiltration. The temperature dependence of the birefringence, dB(m)/dT, was found to be constant of ∼-3.06×10(-6)/K regardless of the magnitude of the pressure.

Charcot-Marie-Tooth (CMT) is a group of clinically and genetically heterogeneous inherited neuromuscular disorders. At present, more than 30 loci have been reported to be associated with CMT disease; point mutations in the mitofusin 2 (MFN2) gene is one of the most common causes. We studied a Chinese family with CMT disease in which the phenotype of affected individuals varied, and the weakness condition of the distal legs in males, except the proband, was less severe than in females in this family. Linkage analysis and PCR sequencing revealed a missense mutation (NM&#95;014874.3:c.1066 A>G) in the MFN2 gene, resulting in an animo acid substitution of threonine to alanine in condon 356 (Thr356Ala). This is a novel phenotype and mutation for CMT family.

The dispersion-shifted fiber (DSF) incorporated with Si nanocrystals (Si-NCs) having highly nonlinear optical property was fabricated to investigate the effective supercontinuum generation characteristics by using the MCVD process and the drawing process. Optical nonlinearity was enhanced by incorporating Si nanocrystals in the core of the fiber and the refractive index profile of a dispersion-shifted fiber was employed to match its zero-dispersion wavelength to that of the commercially available pumping source for generating effective supercontinuum. The non-resonant nonlinear refractive index, n2, of the Si-NCs doped DSF measured by the cw-SPM method was measured to be 7.03 x 10(-20) [m2/W] and the coefficient of non-resonant nonlinearity, gamma, was 7.14 [W(-1) km(-1)]. To examine supercontinuum generation of the Si-NCs doped DSF, the femtosecond fiber laser with the pulse width of 150 fs (at 1560 nm) was launched into the fiber core. The output spectrum of the Si-NCs doped DSF was found to broaden from 1300 nm to wavelength well beyond 1700 nm, which can be attributed to the enhanced optical nonlinearity by Si-NCs embedded in the fiber core. The short wavelength of the supercontinuum spectrum in the Si-NCs doped DSF showed shift from 1352 nm to 1220 nm for the fiber length of 2.5 m and 200 m, respectively.

Cu/Zn-codoped germano-silicate optical glass fiber was manufactured by using the modified chemical vapor deposition (MCVD) process and solution doping process. To investigate the reduction effect of Zn addition on Cu metal formation in the core of the Cu/Zn-codoped germano-silicate optical glass fiber, the optical absorption property and the non-resonant third-order optical nonlinearity were measured. Absorption peaks at 435 nm and 469 nm in the Cu/Zn-codoped germano-silicate optical glass fiber were contributed to Cu metal particles and ZnO semiconductor particles, respectively. The effective non-resonant optical nonlinearity, gamma, of the Cu/Zn-codoped germano-silicate optical glass fiber was measured to be 1.5097 W(-1) x km(-1) by using the continuous-wave self-phase modulation method. The gamma of the Cu/Zn-codoped germano-silicate optical glass fiber was about four times larger than that of the reference germano-silicate optical glass fiber without any dopants. The increase of the effective non-resonant optical nonlinearity, gamma, of the Cu/Zn-codoped germano-silicate optical glass fiber, can be attributed to the enhanced nonlinear polarization due to incorporated ZnO semiconductor particles and Cu metal ions in the glass network. The Cu/Zn-codoped germano-silicate optical glass fiber showed high nonlinearity and low transmission loss at the optical communication wavelength, which makes it suitable for high-speed-high-capacity optical communication systems.

The analog mean-delay (AMD) method is a new alternative method to measure the lifetime of a fluorescence molecule. Because of its powerful advantages of accurate lifetime determination, good photon economy, and a high photon detection rate, the AMD method is considered to be very suitable for high-speed confocal fluorescence lifetime imaging microscopy (FLIM). For the practical usage of the AMD method in FLIM (AMD-FLIM), detailed study on various experimental conditions and parameters that affect the precision and the accuracy of the AMD method is required. In this paper, we present the relation between the precision and accuracy of the lifetime versus iteration number in the AMD method, the best cutoff frequency of a low-pass filter used in the AMD-FLIM system for a given fluorophore, and the optimum position and width of the integration window by using Monte Carlo simulations and a series of AMD-FLIM experiments.

We demonstrate a high-speed confocal fluorescence lifetime imaging microscopy (FLIM) whose accuracy and photon economy are as good as that of a time-correlated single photon counting (TCSPC). It is based on a new lifetime determination scheme, the analog mean delay (AMD) method. Due to the technical advantages of multiple fluorescence photon detection capability, accurate lifetime determination scheme and high photon detection efficiency, the AMD method can be the most effective method for high-speed confocal FLIM. The feasibility of real-time confocal FLIM with the AMD method has been demonstrated by observing the dynamic reaction of calcium channels in a RBL-2H3 cell with respect to 4αPDD stimulus. We have achieved the photon detection rate of 125 times faster than a conventional TCSPC based system in this experiment.

Germano-silicate glass optical fiber incorporated with PbTe semiconductor quantum dots (SQDs) in the core was fabricated by using the atomization process in modified chemical vapor deposition (MCVD) process. The absorption bands attributed to PbTe semiconductor quantum dots in the fiber core were found to appear at around 687 nm and 1055 nm. The nonlinear refractive index measured by the long-period fiber grating (LPG) pair method upon pumping with laser diode at 976.4 nm was estimated to be ~1.5 × 10(-16) m2/W.

The analog mean-delay (AMD) method is a new powerful alternative method in determining the lifetime of a fluorescence molecule for high-speed confocal fluorescence lifetime imaging microscopy. Even though the photon economy and the lifetime precision of the AMD method are proven to be as good as those of the state-of-the-art time-correlated single photon counting method, there have been some speculations and concerns about the accuracy of this method with respect to the absolute lifetime value of a fluorescence probe. In the AMD method, the temporal waveform of an emitted fluorescence signal is directly recorded with a slow digitizer whose bandwidth is much lower than the temporal resolution of the lifetime to be measured. We have found that the drifts and the fluctuations of the absolute zero position in a measured temporal waveform are the major problems in the AMD method. We have proposed electrical and optical referencing techniques that may suppress these errors. It is shown that there may exist more than 2 ns drift in a measured temporal waveform during the period of the first 12 min after electronic components are turned on. The standard deviation of a measured lifetime after this warm-up period can be as large as 51 ps without any referencing technique. We have shown that this error can be reduced to 9 ps with our electronic referencing technique. It is demonstrated that this can be further reduced to 4 ps by the optical referencing technique we have introduced.

We have experimentally developed a highly sensitive and a compact size current sensor by using the CdSe quantum dots-doped bend insensitive optical fiber, operating in the visible band of wavelength. The modified sensitivity of this sensor was about 675 microrad/(Turn.A.m) for the loop radius of just 10 mm, which is more than 16 times larger than that of the single mode optical fiber current sensor.

We present a simple and reliable method based on the spectral splice loss measurement to determine the cutoff wavelength of bend insensitive fiber.

We observed intense green upconversion emission from Tb(3+) centered at 546 nm due to transition D(4)5-->F(5)7 in Tb(3+)/Yb(3+) codoped alumino-germano-silicate optical fibers by direct excitation of Yb(3+) ions with a laser diode at 976 nm. A two-photon cooperative energy transfer upconversion among a pair of Yb(3+) donor ions and a Tb(3+) acceptor ion are responsible for the observed green emission. Appropriately increasing the concentration of Tb(3+) relative to Yb(3+) was found to facilitate the energy transfer upconversion process from Yb(3+) to Tb(3+), while an excess of Tb(3+) ions inversely introduced more of the concentration quenching effect and phonon-assistant backward energy transfer from Tb(3+) to Yb(3+) and, therefore, damaged the observed green emission. The intensity of the observed green emission was found optimum for a concentration ratio of Tb(3+) to Yb(3+) of 2:1 in alumino-germano-silicate optical glass fibers.

We report experimental development of a single-mode optical fiber optimized for a wavelength band of 400 nm to 700 nm, which showed negligible bending loss of 0.09 dB/loop at 400 nm and 0.23 dB/loop at 633 nm for a loop diameter of 10 mm.

We have developed a new single-mode optical fiber (SMF) which exhibits ultra low bend sensitivity over a wide communication band. The measured mean bending loss at 1550 nm was about 0.0095 dB for a loop of 10 mm diameter.

A tellurium-doped germanosilicate optical fiber was developed by modified chemical vapor deposition and solution doping techniques. Using the continuous-wave self-phase modulation method, the nonresonant nonlinear refractive index, n2, was measured to be 5.52 x 10(-20) m2/W, which is 2 to 3 times that of the undoped germanosilicate glass fiber. Polyhedron structures TeO3 and TeO4 with GeO4 and SiO4 are believed responsible for lower phonon energy, more nonbridging oxygens, and a larger hyperpolarization, leading to the observed higher nonresonant optical nonlinearity.

We have designed and experimentally demonstrated a single mode optical fiber with a very low bending loss of about 0.014 dB/loop at 1550 nm for a bending radius of 5 mm.

Effect of filler metals (Bi, 80Au-20Sn alloy, Sn, and In) on the temperature sensitivity of a birefringent side-hole fiber was investigated. The temperature sensitivity of the fiber was found to increase by introduction of the filler metals and the sensitivity gradually increased with the increase of the thermal expansion coefficient of metals. The sensitivity was larger for the filler metals with the larger thermal expansion coefficient.

Ag-nanocrystal-incorporated germano-silicate optical fiber with high resonant nonlinearity was fabricated by using modified chemical vapor deposition and solution doping techniques. An all-optical signal switching application based on cross-phase modulation was demonstrated in the cascaded long-period fiber gratings. Pumped with 499 nm argon laser, all-optical signal gating of the pi-phase shift was achieved with low pump intensities of less than 7.64 GW/cm2.

A new optical fiber current sensor using a CdSe quantum dots doped optical fiber has been demonstrated with high Faraday rotation for remote sensing of current from 0 to 40 Amperes. It showed enhancement in the current sensitivity by about 2 times than that of the single mode optical fiber current sensor at 632 nm.