Results 1 to 10 of about 4,249 (178)

Polymorphism of the 86th amino acid in CX26 protein and hereditary deafness [PDF]

Journal of Otology, 2016
Objective: To investigate the membrane localization function of the CX26 protein when its 86th amino acid is Thr, Ser or Arg, and its relations to deafness.
Shiming Yang, Yuehua Qiao
exaly   +6 more sources

Connexin 26 Functions as a Direct Transcriptional Regulator During the Cochlea Development [PDF]

Advanced Science
Gap junction Beta 2 Protein (GJB2, Connexin26, Cx26), the primary genetic cause of hereditary hearing loss (25%–50% of cases), has been exclusively regarded as forming an intercellular channel that mediates rapid communication.
Xiaozhou Liu, Le Xie, Yuan Jin, Sen Chen, Xinyu Shi, Yanjun Zong, Zhengdong Zhao, Wei‐Jia Kong, Yu Sun   +8 more
doaj   +3 more sources

CX26 promotes pancreatic cancer progression by competitively inhibiting interaction of c-Myc with PSMD2 and enhancing c-Myc stability [PDF]

Journal of Translational Medicine
Background Pancreatic cancer (PC) is highly aggressive and fatal and has dismal prognostic outcomes, primarily due to its late-stage diagnosis and limited effective treatments. The molecular mechanisms triggering PC progression are largely unclear.
Cheng He, Chuanyu Tang, Jie Guo, Xiangyi Yin, Qing Zhu, Chihua Fang   +5 more
doaj   +3 more sources

Viral‐Mediated Connexin 26 Expression Combined with Dexamethasone Rescues Hearing in a Conditional Gjb2 Null Mice Model [PDF]

Advanced Science
GJB2 encodes connexin 26 (Cx26), the most commonly mutated gene causing hereditary non‐syndromic hearing loss. Cx26 is mainly expressed in supporting cells (SCs) and fibrocytes in the mammalian cochlea.
Xiaohui Wang, Li Zhang, Sen Chen, Le Xie, Yue Qiu, Chenyang Kong, Ge Yin, Weijia Kong, Yu Sun   +8 more
doaj   +3 more sources

Triggered Calcium Lightning Programs Cochlear Development. [PDF]

Exploration (Beijing)
Summary: Before the onset of hearing, the developing inner ear generates spontaneous calcium signals that are thought to guide maturation. In this study, we discovered a rapid and widespread calcium flash—dubbed “Ca2+ lightning”—originating from supporting cells beneath the sensory hair cells, which triggers coordinated calcium waves across the entire ...
Ma Q, Wang HP, Jiang YM, Lai K, Li MX, Zhang Z, Gong LN, Lai JW, Su KM, Wang LY, Wang H, Yin SK, He H, Shi HB.   +13 more
europepmc   +2 more sources

The Reduction in Microtubule Arrays Caused by the Dysplasia of the Non-Centrosomal Microtubule-Organizing Center Leads to a Malformed Organ of Corti in the Cx26-Null Mouse

Biomedicines, 2022
Mutations in the GJB2 gene account for approximately 20–50% of all non-syndromic hereditary deafness cases. The malformed organ of Corti (OC) was observed in different Cx26-null mouse models, which was mainly caused by the developmental arrest of pillar ...
Yue Qiu, Yu Sun, Sen Chen
exaly   +3 more sources

Cochlear endoplasmic reticulum stress causes connexin 26 degradation is involved in age-related hearing loss [PDF]

iScience
Summary: Age-related hearing loss (ARHL) represents a progressive auditory disorder. Growing evidence indicates that degradation of connexin 26 (Cx26) in cochlear may constitute one of the pathogenic mechanisms in ARHL.
Xue Bai, Bing Liao, Wen-Hui Hu, Mei-Qun Wang, Yu Sun, Xu-Bo Chen, Kai Xu   +6 more
doaj   +2 more sources

Prenatal Diagnosis of Keratitis-Ichthyosis-Deafness Syndrome With Dandy Walker Malformation: A Case Report. [PDF]

Case Rep Obstet Gynecol
Background Keratitis‐ichthyosis‐deafness (KID) syndrome is a rare disorder characterized by progressive vascularizing keratitis, ichthyosiform erythrokeratoderma, and neurosensory hearing loss. It is caused by missense mutations in the GJB2 gene. Its known association with Dandy‐Walker malformation (DWM), a developmental anomaly of the posterior ...
Bourdil L, Georgia Blume C.
europepmc   +2 more sources

Turn-Taking and Vocal Coordination in Mother-Child Mixed-Hearing Dyads and the Effect of Home Music Engagement: A Longitudinal Study on Italian Children With Cochlear Implants. [PDF]

Int J Lang Commun Disord
ABSTRACT Objectives Studies have shown that the amount of turn‐taking and vocal coordination in mother–child dyads, as well as amount of home music engagement, have important effects on children's language and social development. However, relatively few studies have examined the development of vocal behaviours in mixed‐hearing and hearing dyads, and no
Persici V, Guerzoni L, Cuda D, Majorano M.   +3 more
europepmc   +2 more sources

Connexin Regulation and Modulation of Neural Stem Cell Differentiation Induced by Cell-Permeable Itaconate. [PDF]

J Cell Physiol
The immunometabolite itaconate modulates the connexin profile of neural stem cells, and it upregulates Cx36‐based channels, promoting the differentiation toward a neuronal phenotype. These effects were abolished by connexin blockade, supporting a role for itaconate in the neuroimmune axis in inflammatory and neurodegenerative disorders. ABSTRACT Neural
Denaro S, Spina SR, D'Aprile S, Gervasi A, Torrisi F, Parenti C, Zappalà A, Vicario N, Parenti R.   +8 more
europepmc   +2 more sources