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Ionically Imprinting-Based Copper (Ⅱ) Label-Free Detection for Preventing Hearing Loss Article
Huan Wang, Hui Zhang, Xiaoli Zhang, Hong Chen, Ling Lu, Renjie Chai
《工程(英文)》 doi: 10.1016/j.eng.2023.09.001
Copper is a microelement with important physiological functions in the body. However, the excess copper ion (Cu2+) may cause severe health problems, such as hair cell apoptosis and the resultant hearing loss. Therefore, the assay of Cu2+ is important. We integrate ionic imprinting technology (IIT) and structurally colored hydrogel beads to prepare chitosan-based ionically imprinted hydrogel beads (IIHBs) as a low-cost and high-specificity platform for Cu2+ detection. The IIHBs have a macroporous microstructure, uniform size, vivid structural color, and magnetic responsiveness. When incubated in solution, IIHBs recognize Cu2+ and exhibit a reflective peak change, thereby achieving label-free detection. In addition, benefiting from the IIT, the IIHBs display good specificity and selectivity and have an imprinting factor of 19.14 at 100 lmol∙L–1. These features indicated that the developed IIHBs are promising candidates for Cu2+ detection, particularly for the prevention of hearing loss.
关键词: Structural color Microfluidics Ionic imprinting Label-free detection Hearing loss
Research progress in pathogenic genes of hereditary non-syndromic mid-frequency deafness
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《医学前沿(英文)》 2016年 第10卷 第2期 页码 137-142 doi: 10.1007/s11684-016-0449-8
Hearing impairment is considered as the most prevalent impairment worldwide. Almost 600 million people in the world suffer from mild or moderate hearing impairment, an estimated 10% of the human population. Genetic factors play an important role in the pathogenesis of this disorder. Hereditary hearing loss is divided into syndromic hearing loss (associated with other anomalies) and non-syndromic hearing loss (not associated with other anomalies). Approximately 80% of genetic deafness is non-syndromic. On the basis of the frequency of hearing loss, hereditary non-syndromic hearing loss can be divided into high-, mid-, low-, and total-frequency hearing loss. An audiometric finding of mid-frequency sensorineural hearing loss, or a “bowl-shaped” audiogram, is uncommon. Up to now, merely 7 loci have been linked to mid-frequency hearing loss. Only four genetic mid-frequency deafness genes, namely, DFNA10 (EYA4), DFNA8/12 (TECTA), DFNA13 (COL11A2), DFNA44 (CCDC50), have been reported to date. This review summarizes the research progress of the four genes to draw attention to mid-frequency deafness genes.
关键词: hereditary non-syndromic hearing loss mid-frequency hearing loss deafness genes
Alternative splicing of inner-ear-expressed genes
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《医学前沿(英文)》 2016年 第10卷 第3期 页码 250-257 doi: 10.1007/s11684-016-0454-y
Alternative splicing plays a fundamental role in the development and physiological function of the inner ear. Inner-ear-specific gene splicing is necessary to establish the identity and maintain the function of the inner ear. For example, exon 68 of Cadherin 23 (Cdh23) gene is subject to inner-ear-specific alternative splicing, and as a result, Cdh23(+68) is only expressed in inner ear hair cells. Alternative splicing along the tonotopic axis of the cochlea contributes to frequency tuning, particularly in lower vertebrates, such as chickens and turtles. Differential splicing of Kcnma1, which encodes for the α subunit of the Ca2+-activated K+ channel (BK channel), has been suggested to affect the channel gating properties and is important for frequency tuning. Consequently, deficits in alternative splicing have been shown to cause hearing loss, as we can observe in Bronx Waltzer (bv) mice and Sfswap mutant mice. Despite the advances in this field, the regulation of alternative splicing in the inner ear remains elusive. Further investigation is also needed to clarify the mechanism of hearing loss caused by alternative splicing deficits.
《医学前沿(英文)》 doi: 10.1007/s11684-023-1006-x
关键词: neurodevelopmental disorder autosomal recessive intellectual disability consanguinity spastic paraplegia hearing loss TMEM141
Loss of liver kinase B1 causes planar polarity defects in cochlear hair cells in mice
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《医学前沿(英文)》 2016年 第10卷 第4期 页码 481-489 doi: 10.1007/s11684-016-0494-3
The tumor suppressor gene liver kinase B1 (LKB1), also called STK11, encodes a serine/threonine kinase. LKB1 plays crucial roles in cell differentiation, proliferation, and polarity. In this study, LKB1 conditional knockout mice (LKB1Pax2 CKO mice) were generated using Pax2-Cre mice to investigate the function of LKB1 in inner ear hair cells during early embryonic period. LKB1Pax2 CKO mice died perinatally. Immunofluorescence and scanning electron microscopy revealed that stereociliary bundles in LKB1Pax2 CKO mice were clustered and misoriented, respectively. Moreover, ectopic distribution of kinocilium bundles resulting from abnormal migration of kinocilium was observed in the mutant mice. The orientation of stereociliary bundles and the migration of kinocilia are critical indicators of planar cell polarity (PCP) of hair cells. LKB1 deficiency in LKB1Pax2 CKO mice thus disrupted hair cell planar polarity during embryonic development. Our results suggest that LKB1 is required in PCP formation in cochlear hair cells in mice.
关键词: LKB1 stereociliary bundles kinocilium planar cell polarity hearing mice
is essential for the integrity of stereociliary rootlet in cochlear hair cells in mice
Yuqin Men, Xiujuan Li, Hailong Tu, Aizhen Zhang, Xiaolong Fu, Zhishuo Wang, Yecheng Jin, Congzhe Hou, Tingting Zhang, Sen Zhang, Yichen Zhou, Boqin Li, Jianfeng Li, Xiaoyang Sun, Haibo Wang, Jiangang Gao
《医学前沿(英文)》 2019年 第13卷 第6期 页码 690-704 doi: 10.1007/s11684-018-0638-8
关键词: TPRN stereocilia stereociliary rootlet actin filament CRISPR/Cas9 hearing
标题 作者 时间 类型 操作
Ionically Imprinting-Based Copper (Ⅱ) Label-Free Detection for Preventing Hearing Loss
Huan Wang, Hui Zhang, Xiaoli Zhang, Hong Chen, Ling Lu, Renjie Chai
期刊论文
Panoramic variation analysis of a family with neurodevelopmental disorders caused by biallelic loss-of-function variants in , , and
期刊论文