Degradation of cochlear Connexin26 accelerate the development of age-related hearing loss.

The GJB2 gene, encoding Connexin26 (Cx26), is one of the most common causes of inherited deafness. Clinically, mutations in GJB2 cause congenital deafness or late-onset progressive hearing loss. Recently, it has been reported that Cx26 haploid deficiency accelerates the development of age-related hearing loss (ARHL).

Automatic Detection and Association Analysis of Multiple Surface Defects on Shield Subway Tunnels.

The surface defects on a shield subway tunnel can significantly affect the serviceability of the tunnel structure and may compromise operation safety. To effectively detect multiple surface defects, this study uses a tunnel inspection trolley (TIT) based on the mobile laser scanning technique. By conducting an inspection of the shield tunnel on a metro line section, various surface defects are identified with the TIT, including water leakage defects, dislocation, spalling, cross-section deformation, etc.

Cytomembrane Trafficking Pathways of Connexin 26, 30, and 43.

The connexin gene family is the most prevalent gene that contributes to hearing loss. Connexins 26 and 30, encoded by and , respectively, are the most abundantly expressed connexins in the inner ear. Connexin 43, which is encoded by , appears to be widely expressed in various organs, including the heart, skin, the brain, and the inner ear.

Targeted Next-Generation Sequencing Identified Novel Compound Heterozygous Variants in the Gene Causing Autosomal Recessive Hearing Loss in a Chinese Family.

Hearing loss is among the most common congenital sensory impairments. Genetic causes account for more than 50% of the cases of congenital hearing loss. The gene, encoding protein tyrosine phosphatase receptor Q, plays an important role in maintaining the stereocilia structure and function of hair cells.

The protective effects of systemic dexamethasone on sensory epithelial damage and hearing loss in targeted Cx26-null mice.

Mutations in the GJB2 gene (encoding Connexin26(Cx26)) are the most common cause of hereditary deafness, accounting for about a quarter of all cases. Sensory epithelial damage is considered to be one of the main causes of deafness caused by GJB2 gene mutation. Dexamethasone (DEX) is widely used in the treatment of a variety of inner ear diseases including sudden sensorineural hearing loss (SSNHL), noise-induced hearing loss (NIHL), and deafness caused by ototoxic drugs.

F-Actin Dysplasia Involved in Organ of Corti Deformity in Knockdown Mouse Model.

Mutations in the gene encoding connexin26 (Cx26) protein are one of the most common causes of hereditary deafness. Previous studies have found that different Cx26-null mouse models have severe hearing loss and deformity of the organ of Corti (OC) as well as a reduction in microtubules in pillar cells (PCs). To explore the underlying mechanism of OC deformity caused by Cx26 downregulation further, we established Cx26 knockdown (KD) mouse models at postnatal days (P)0 and P8.

Sensitive osteosarcoma diagnosis through five-base telomerase product-triggered CRISPR-Cas12a enhanced rolling circle amplification.

Osteosarcoma is the most frequent primary malignant bone tumor, composed of mesenchymal cells producing osteoid and immature bone. The sensitive detection of telomerase plays a pivotal role in the early diagnosis and therapeutic treatment of osteosarcoma. We report here an strategy for sensitive telomerase activity detection through the integration of rolling circle amplification (RCA) and a clustered regularly spaced short palindrome repeats (CRISPR)-Cas12a system.

Hearing Screening Combined with Target Gene Panel Testing Increased Etiological Diagnostic Yield in Deaf Children.

Genetic testing is the gold standard for exploring the etiology of congenital hearing loss. Here, we enrolled 137 Chinese patients with congenital hearing loss to describe the molecular epidemiology by using 127 gene panel testing or 159 variant testing. Sixty-three deaf children received 127 gene panel testing, while seventy-four patients received 159 variant testing.

Identification of Novel Compound Heterozygous Mutations in Two Chinese Families with Autosomal Recessive Nonsyndromic Hearing Loss.

Congenital deafness is one of the most common causes of disability in humans, and more than half of cases are caused by genetic factors. Mutations of the gene are the third most common cause of hereditary hearing loss. Using next-generation sequencing combined with auditory tests, two novel compound heterozygous variants c.

N-Acetylcysteine Combined With Dexamethasone Treatment Improves Sudden Sensorineural Hearing Loss and Attenuates Hair Cell Death Caused by ROS Stress.

Sudden sensorineural hearing loss (SSNHL) is a common emergency in the world. Increasing evidence of imbalance of oxidant-antioxidant were found in SSNHL patients. Steroids combined with antioxidants may be a potential strategy for the treatment of SSNHL.

Local Macrophage-Related Immune Response Is Involved in Cochlear Epithelial Damage in Distinct -Related Hereditary Deafness Models.

The macrophage-related immune response is an important component of the cochlear response to different exogenous stresses, including noise, ototoxic antibiotics, toxins, or viral infection. However, the role of the immune response in hereditary deafness caused by genetic mutations is rarely explored. , encoding connexin 26 (Cx26), is the most common deafness gene of hereditary deafness.

Surface Crack Detection in Precasted Slab Track in High-Speed Rail via Infrared Thermography.

The surface crack of ballastless track slab can seriously reduce the serviceability and durability of high-speed railway (HSR). Aiming at accurately and efficiently detecting the slab cracks, this research proposes an infrared thermography (IRT)-based method for the surface crack, which is the most serious and common crack type in track slab. A three dimensional finite element (FE) model of IRT detection of concrete slab with surface cracks is established.

A Novel Spontaneous Mutation of the Gene Associated with Waardenburg Syndrome Type II.

Waardenburg syndrome (WS), also known as auditory-pigmentary syndrome, is the most common cause of syndromic hearing loss. It is responsible for 2-5% of congenital deafness. WS is classified into four types depending on the clinical phenotypes.

Jervell and Lange-Nielsen Syndrome due to a Novel Compound Heterozygous Mutation in a Chinese Family.

Jervell and Lange-Nielsen syndrome (JLNS) is a rare but severe autosomal recessive disease characterized by profound congenital deafness and a prolonged QTc interval (greater than 500 milliseconds) in the ECG waveforms. The prevalence of JLNS is about 1/1000000 to 1/200000 around the world. However, exceed 25% of JLNS patients suffered sudden cardiac death with kinds of triggers containing anesthesia.

Identification of Temperature-Induced Deformation for HSR Slab Track Using Track Geometry Measurement Data.

Slab track is widely used in many newly built high-speed rail (HSR) lines as it offers many advantages over ballasted tracks. However, in actual operation, slab tracks are subjected to operational and environmental factors, and structural damages are frequently reported. One of the most critical problems is temperature-induced slab-warping deformation (SWD) which can jeopardize the safety of train operation.

Auditory Neuropathy Spectrum Disorder due to Two Novel Compound Heterozygous Mutations in Two Chinese Families.

Auditory neuropathy spectrum disorder (ANSD), also called auditory neuropathy (AN), is a unique type of prelingual hearing impairment. Up to 10% of deaf infants and children are affected by this disease. Mutation of the gene which encodes otoferlin is the common cause of congenital nonsyndromic ANSD.

Wayside Detection of Wheel Minor Defects in High-Speed Trains by a Bayesian Blind Source Separation Method.

For high-speed trains, out-of-roundness (OOR)/defects on wheel tread with small radius deviation may suffice to give rise to severe damage on both vehicle components and track structure when they run at high speeds. It is thus highly desirable to detect the defects in a timely manner and then conduct wheel re-profiling for the defective wheels. This paper presents a wayside fiber Bragg grating (FBG)-based wheel condition monitoring system which can detect wheel tread defects online during train passage.

Fat-Soluble Vitamin Deficiency in Pediatric Patients with Biliary Atresia.

Objective: To analyze the levels of fat-soluble vitamins (FSVs) in pediatric patients with biliary atresia (BA) before and after the Kasai procedure.

Methods: Pediatric patients with obstructive jaundice were enrolled in this study. The FSV levels and liver function before, 2 weeks after, and 1, 3, and 6 months after the Kasai procedure were measured.

Knockdown of E2F3 Inhibits Proliferation, Migration, and Invasion and Increases Apoptosis in Glioma Cells.

E2F3a, as a member of the E2F family, is essential for cell division associated with the progression of many cancers. However, the biological effect of E2F3a on glioma is not understood as well. To investigate the functional mechanism of E2F3a in glioma, we examined the expression of E2F3a in glioma tissue and cell lines.

Inhibition of Growth and Metastasis of Tumor in Nude Mice after Intraperitoneal Injection of Bevacizumab.

Objective: To explore the inhibitory effect of bevacizumab, a vascular endothelial growth factor antibody, on angiogenesis in human osteosarcoma of nude mice.

Methods: Twenty-one nude mice were inoculated with red fluorescent protein (RFP)-labeled human osteosarcoma cell line 143B-RFP, that is, clones that expressed RFP in the cytoplasm, and randomly assigned to one of three groups: G1 (Control group, injected with saline solution); G2 (intraperitoneal bevacizumab 2 mg/kg twice per week) and G3 (intraperitoneal bevacizumab 5 mg/kg, twice per week). The tumor-bearing mice were examined in a fluorescence light box that was illuminated periodically.

Expression of Matrix Metalloproteinase-9 and CD34 in Giant Cell Tumor of Bone.

Objective: Giant cell tumor of bone (GCTB) invades extensively and metastasizes, however, the pathological grade and imaging findings are not accurate predictors of its prognosis. Thus, the aim of this study was to explore the relationships between expression of cluster of differentiation (CD)34 and matrix metalloproteinase-9 (MMP-9) and the biological behavior of GCTB with the hope of identifying predictors of prognosis.

Methods: Sixty-eight patients with GCTBs attending our institution from September 2008 to August 2013 were enrolled in this prospective study and grouped according to tumor location.

Involvement of α5 integrin in survivin-mediated osteosarcoma metastasis.

Objective: To investigate the role of survivin in osteosarcoma metastasis.

Methods: Small interfering RNA (siRNA) was used to knockdown the expression of survivin and α5 integrin in the human osteosarcoma cell line MG63. Western blotting and immunostaining methods were used to assessed the effect of survivin knockdown on the expression of α5 integrin through flow cytometry and fluorescence microscopy detection.

Effect of REAu on Silver Staining Results and Their UV-Vis Absorption Spectra.

La, Ce, Nd, Sm, Eu, Gd, Dy particles and gold nanoparticles were prepared. Effects of La-Au, Ce-Au, Nd-Au, Sm-Au, Eu-Au, Gd-Au and Dy-Au particles on silver staining results were studied, respectively, and UV-Vis absorption spectra of La-Au, Ce-Au, Nd-Au, Sm-Au, Eu-Au, Gd-Au and Dy-Au particles were studied. Times and colors of sports with La-Au, Ce-Au, Nd-Au, Sm-Au, Eu-Au, Gd-Au, Dy-Au particles are longer and darker than that of with gold nanoparticles, respectively.

Effect of Heat Treatment Temperature on the Spectral Properties of Cu-Ni Coating.

Cu-Ni coatings were prepared on the surface of nickel by electrodeposition method, and Cu-Ni coatings were heat-treated in 25-900 °C. Heat-treated Cu-Ni coatings were characterized with scanning electron microscopy (SEM), energy dispersive x-ray analysis (EDAX) and X-ray diffraction (XRD) techniques, respectively. Effects of heat treatment temperature on the spectral properties of Cu-Ni coatings were studied.