Results 341 to 350 of about 10,050,443 (381)

RONIN/HCF1‐TFEB Axis Protects Against D‐Galactose‐Induced Cochlear Hair Cell Senescence Through Autophagy Activation

Advanced Science, EarlyView.
D‐galactose (D‐gal) induced inner ear hair cell senescence by inhibiting TFEB transcription. RONIN/HCF1 promotes TFEB transcription to prevent cochlear HCs from D‐gal‐induced senescence through autophagy activation. Abstract Age‐related hearing loss is characterized by senescent inner ear hair cells (HCs) and reduced autophagy.
Yongjie Wei, Yuhua Zhang, Wei Cao, Nan Cheng, Yun Xiao, Yongjun Zhu, Yan Xu, Lei Zhang, Lingna Guo, Jun Song, Su‐hua Sha, Buwei Shao, Fang Ma, Jingwen Yang, Zheng Ying, Zuhong He, Renjie Chai, Qiaojun Fang, Jianming Yang   +18 more
wiley   +1 more source

Elevated Histone Lactylation Mediates Ferroptosis Resistance in Endometriosis Through the METTL3‐Regulated HIF1A/HMOX1 Signaling Pathway

Advanced Science, EarlyView.
This study reveals that histone lactylation, a modification derived from lactate, plays a crucial role in ferroptosis resistance in ectopic endometrial stromal cells. This resistance is mediated via the HIF1A/HMOX1 pathway regulated by METTL3. In vivo, a combination of 2‐DG and erastin shows promise as an effective treatment for endometriosis ...
Zongwen Liang, Jinming Liu, Yanling Gou, Honglin Wang, Zhi Li, Yingying Cao, Huiyan Zhang, Ruru Bai, Zongfeng Zhang   +8 more
wiley   +1 more source

Exploring AAV‐Mediated Gene Therapy for Inner Ear Diseases: from Preclinical Success to Clinical Potential

Advanced Science, EarlyView.
Current preclinical studies of AAV‐mediated gene therapy explore different strategies based on the characteristics of inner ear diseases. For genetic hearing loss, approaches include the replacement of a “good gene,” removal of a “bad gene,” or direct correction of mutations through base editing.
Fan Wu, Wuhui He, Yun Xiao, Qiong Wu, Qing Zhang, Tiesong Zhang, Lei Xu, Xinyi Pang   +7 more
wiley   +1 more source

Pinoresinol Diglucoside Attenuates Nuclear Receptor Coactivator 4‐Mediated Ferritinophagy Associated with Cisplatin‐Induced Hearing Loss

Advanced Science, EarlyView.
Pinoresinol diglucoside, a bioactive component extracted from traditional Chinese medicine, attenuates cisplatin‐induced ototoxicity in vivo and in vitro. The protective effect is achieved by downregulation of Socs1. The present study is the first to investigate the expression and function of Socs1 in inner ear, which is known to promote ferroptosis ...
Yin Chen, Cheng Cheng, Ao Li, Dengbin Ma, Siyu Li, Handong Wang, Song Gao, Dingding Liu, Panpan Song, Chenjie Yu, Xiaoyun Qian, Guoqiang Wan, Xia Gao   +12 more
wiley   +1 more source

A Multifunctional Nanodelivery System Modified by Fusion Peptides Acts as Teriparatide Carrier for Noise‐Induced Hearing Loss Therapy

Advanced Science, EarlyView.
The fusion peptide LR27‐modified thermosensitive nanodelivery system exhibits both hair cell targeting and inner ear penetrating properties. This system sustainably and effectively delivers PTH1‐34 to the inner ear of a hearing loss mouse model via the synergistic effects of multiple peptides, achieving satisfactory hearing protection through ...
Jiawen Li, Zhuowen Hao, Fangzi Ke, Zhihui Liu, Weilong Wang, Sihui Wen, Fan Wu, Bowen Xu, Miao He, Shengyu Zou, Xiong Chen, Jingfeng Li, Zuhong He   +12 more
wiley   +1 more source

NOX2 Contributes to High‐Frequency Outer Hair Cell Vulnerability in the Cochlea

Advanced Science, EarlyView.
This study first identifies NOX2 as a differentially expressed gene related to oxidative damage in the apical and basal turns through single‐cell RNA sequencing. NOX2 gene knockout mitigates OHCs damage caused by neomycin and noise and enhances Nrf2 expression and nuclear translocation.
Meihao Qi, Zejun Gao, Yang Qiu, Renfeng Wang, Keyong Tian, Bo Yue, Xinyu Zhang, Peng Zhang, Ziqi Wu, Qingwen Zhu, Zhenzhen Liu, Zhuoyao Ma, Xueying Zhou, Yu Han, Jun Chen, Jianhua Qiu, DingJun Zha   +16 more
wiley   +1 more source

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Genetic basis of mitochondrial diseases

FEBS Letters, 2021
Mitochondrial disorders are monogenic disorders characterized by a defect in oxidative phosphorylation and caused by pathogenic variants in one of over 340 different genes.
Mirjana Gusic, H. Prokisch
semanticscholar   +1 more source

Mitochondrial diseases

Neurologic Clinics, 2002
Since the first reports of disorders associated with mitochondrial DNA (mtDNA) defects more than a decade ago, the small mtDNA circle has been a Pandora's box of pathogenic mutations associated with human diseases. The "morbidity map" of mtDNA has gone from one point mutation and a few deletions in 1988 to more than 110 point mutations as of September,
Tuan H, Vu, Michio, Hirano, Salvatore, DiMauro   +2 more
openaire   +4 more sources

Mitochondrial disease

Current Treatment Options in Neurology, 2001
Mitochondrial diseases are disorders of energy metabolism that include defects of pyruvate metabolism, Krebs cycle, respiratory chain (RC), and fatty acid oxidation (FAO). Treatment of pyruvate metabolism, Krebs cycle, and RC disorders is, in general, disappointing.
Roser Pons, Darryl C. De Vivo
openaire   +3 more sources

Mitochondrial diseases

2018
Mitochondrial diseases collectively describe a diverse group of heritable disorders that invariably affect mitochondrial respiratory chain function and cellular energy production. Together they represent the most common cause of inherited metabolic disease, may present at any age, have a wide spectrum of clinical manifestations, may be insidious in ...
Ryan L, Davis, Christina, Liang, Carolyn M, Sue   +2 more
openaire   +5 more sources