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2026

邻近标记技术对活体小鼠耳蜗毛细胞表面蛋白进行特异性标记

王暖，王欣阳，吉长皓，刘敏，高建刚，孙锦

《山东大学耳鼻喉眼学报》

2026

Tox regulates hair cell stereocilia development and Cdh23 expression in mice and zebrafish.

Jing Zhang, Jie Gong, Jing Zhou, Gui-Yi Zhang, Shengda Cao, Si-Wei Guo, Yu Xiao, Xiao-Xu Zhao, Wen Li, Yuhan Wang, Rui-Feng Qiao, Min Wang, Zi-Yi Liu, Guo-Dong Hong, Yun-Hao Wu, Xiu-Li Bi, Hailong Tu, Shu-Yuan Shen, Jiangang Gao, Dong Liu, Xiao-Long Fu

Proceedings of the National Academy of Sciences of the United States of America

精选作品

期刊论文

邻近标记技术对活体小鼠耳蜗毛细胞表面蛋白进行特异性标记

Tox regulates hair cell stereocilia development and Cdh23 expression in mice and zebrafish.

Tmem67 Is Required for Spermiogenesis and Male Fertility in Mice

GSDMD-mediated mitochondrial dysfunction in marginal cells: A potential driver of inflammation and stria vascularis damage in CIHL

Inhibitors of the ubiquitin-proteasome system rescue cellular levels and ion transport function of pathogenic pendrin (SLC26A4) protein variants

TMEM138: From Biological Functions to Diseases

Loxl3 Affects Palatal Shelf Elevation by Regulating Cell Proliferation and Collagen Deposition

mRNA metabolism regulator human antigen R (HuR) regulates age-related hearing loss in aged mice

Author Correction: mRNA metabolism regulator human antigen R (HuR) regulates age-related hearing loss in aged mice

TNFAIP8L2 maintains hair cell function and regulates age-related hearing loss via mTORC1 signaling

Loss of Cep135 causes oligoasthenoteratozoospermia and male infertility in mice

Author Correction: Cep78 knockout causes sterility and oligoasthenoteratozoospermia in male mice

Cep78 knockout causes sterility and oligoasthenoteratozoospermia in male mice

Akr7a5缺失导致小鼠精子成熟受损

Gss deficiency causes age-related fertility impairment via ROS-triggered ferroptosis in the testes of mice

RhoGDIα regulates spermatogenesis through Rac1/cofilin/F-actin signaling

Peroxisome Deficiency in Cochlear Hair Cells Causes Hearing Loss by Deregulating BK Channels

Conditional deletion of Hspa5 leads to spermatogenesis failure and male infertility in mice.

Loss of BAF (mSWI/SNF) chromatin-remodeling ATPase Brg1 causes multiple malformations of cortical development in mice.

Gstm1/Gstt1 is essential for reducing cisplatin ototoxicity in CBA/CaJ mice

Activation of Rictor/mTORC2 signaling acts as a pivotal strategy to protect against sensorineural hearing loss

Pre‐meiotic deletion of PEX5 causes spermatogenesis failure and infertility in mice

Cathepsin B plays a role in spermatogenesis and sperm maturation through regulating autophagy and apoptosis in mice

Hadh deficiency induced oligoasthenoteratozoospermia through the TNF‐α/Bcl‐2 pathway in male mice

Progressive Degeneration of the Retina in Loxl3 Mutant Mouse Model of Stickler Syndrome

Targeted Deletion of Loxl3 by Col2a1-Cre Leads to Progressive Hearing Loss

Deficiency of Klc2 Induces Low-Frequency Sensorineural Hearing Loss in C57BL/6 J Mice and Human

Deficiency for Lcn8 causes epididymal sperm maturation defects in mice.

SMPX Deficiency Causes Stereocilia Degeneration and Progressive Hearing Loss in CBA/CaJ Mice

Mechanism and Prevention of Ototoxicity Induced by Aminoglycosides

Piccolo is essential for the maintenance of mouse retina but not cochlear hair cell function

Cdc14a has a role in spermatogenesis, sperm maturation and male fertility.

Elamipretide as a potential candidate for relieving cryodamage to human spermatozoa during cryopreservation.

Lgl1 deficiency disrupts hippocampal development and impairs cognitive performance in mice

Loss of Lgl1 Disrupts the Radial Glial Fiber-guided Cortical Neuronal Migration and Causes Subcortical Band Heterotopia in Mice.

Mito-Tempo alleviates cryodamage by regulating intracellular oxidative metabolism in spermatozoa from asthenozoospermic patients.

A knock-in mouse model of Pendred syndrome with Slc26a4 L236P mutation.

Deletion of Brg1 causes stereocilia bundle fusion and cuticular plate loss in vestibular hair cells.

Elmod3 knockout leads to progressive hearing loss and abnormalities in cochlear hair cell stereocilia.

Oocyte‐specific deletion of Gs&agr; induces oxidative stress and deteriorates oocyte quality in mice

Knock-In Mice with Myo3a Y137C Mutation Displayed Progressive Hearing Loss and Hair Cell Degeneration in the Inner Ear

Tprn is essential for the integrity of stereociliary rootlet in cochlear hair cells in mice

Tuberous sclerosis complex–mediated mTORC1 overactivation promotes age-related hearing loss

Correction: Lgl1 Is Required for Olfaction and Development of Olfactory Bulb in Mice

诱导多能干细胞的制备及其应用进展

Loss of liver kinase B1 causes planar polarity defects in cochlear hair cells in mice

Lgl1 Is Required for Olfaction and Development of Olfactory Bulb in Mice

Abnormal mRNA Splicing but Normal Auditory Brainstem Response (ABR) in Mice with the Prestin (SLC26A5) IVS2-2A>G mutation

Sorting nexin 9 (SNX9) is not essential for development and auditory function in mice

Deletion of Brg1 causes abnormal hair cell planer polarity, hair cell anchorage, and scar formation in mouse cochlea

Loss of Myh14 Increases Susceptibility to Noise-Induced Hearing Loss in CBA/CaJ Mice

Trio gene is required for mouse learning ability.

LKB1 Regulates Cerebellar Development by Controlling Sonic Hedgehog-mediated Granule Cell Precursor Proliferation and Granule Cell Migration

LKB1 Is Required for the Development and Maintenance of Stereocilia in Inner Ear Hair Cells in Mice

Abnormal cerebellar development and Purkinje cell defects in Lgl1-Pax2 conditional knockout mice.

PTEN regulation of the proliferation and differentiation of auditory progenitors through the PTEN/PI3K/Akt-signaling pathway in mice

FCHSD1 and FCHSD2 Are Expressed in Hair Cell Stereocilia and Cuticular Plate and Regulate Actin Polymerization In Vitro

Normal Hearing Sensitivity at Low-to-Middle Frequencies with 34% Prestin-Charge Density

Outer hair cell–specific prestin-CreERT2 knockin mouse lines

Advances of gene knockout in mice

Normal Hearing Sensitivity at Low to Middle Frequencies with ∼25% Prestin

Polycystin-1 is required for stereocilia structure but not for mechanotransduction in inner ear hair cells

A review of current large‐scale mouse knockout efforts

Essential and synergistic roles of RP1 and RP1L1 in rod photoreceptor axoneme and retinitis pigmentosa

Glucose transporter 5 is undetectable in outer hair cells and does not contribute to cochlear amplification.

Prestin-based outer hair cell motility is necessary for mammalian cochlear amplification.

Prestin-based outer hair cell electromotility in knockin mice does not appear to adjust the operating point of a cilia-based amplifier

Orphan Glutamate Receptor δ1 Subunit Required for High-Frequency Hearing

Absence of hearing loss in a mouse model for DFNA17 and MYH9-related disease: the use of public gene-targeted ES cell resources.

The Ciliary Rootlet Maintains Long-Term Stability of Sensory Cilia

The Ciliary Rootlet Provides Structural Support for Photoreceptor Outer Segments

Targeting hearing genes in mice.

Inner hair cell Cre‐expressing transgenic mouse

Hearing threshold elevation precedes hair-cell loss in prestin knockout mice.

Prestin is required for electromotility of the outer hair cell and for the cochlear amplifier

Progressive photoreceptor degeneration, outer segment dysplasia, and rhodopsin mislocalization in mice with targeted disruption of the retinitis pigmentosa-1 (Rp1) gene

会议论文

利用基因敲除小鼠模型验证一新的耳聋基因Tmem132e