Results 91 to 100 of about 48,911 (292)

The proteostasis burden of aneuploidy

Biological Chemistry
Abstract Aneuploidy refers to chromosome number abnormality that is not an exact multiple of the haploid chromosome set. Aneuploidy has largely negative consequences in cells and organisms, manifested as so-called aneuploidy-associated stresses.
Prince Saforo Amponsah, Zuzana Storchová   +1 more
openaire   +2 more sources

PROTEOSTASIS AS A SIGNALING DEVICE AND THERAPEUTIC TARGET

, 2011
The term ‘conformational disorders’ was created recently for the numerous pathological situations due to the accumulation of aberrant proteins (or normal ones in excess).
CENCI S, SITIA , ROBERTO
core  

Functional Modules of the Proteostasis Network [PDF]

, 2019
Cells invest in an extensive network of factors to maintain protein homeostasis (proteostasis) and prevent the accumulation of potentially toxic protein aggregates.
Hipp, M., Jayaraj, G., Hartl, F.
core   +1 more source

CHCHD10 Mitigates Alzheimer's Disease‐Related Phenotypes in Association With Epigenetic Remodeling in Directly Reprogrammed Neurons

Advanced Science, EarlyView.
CHCHD10 loss in Alzheimer's disease is associated with mitochondrial dysfunction, epigenomic disruption, and tau pathology. Restoration of CHCHD10 shifts DNA methylation toward a non‐disease state and reduces tau and amyloid pathology, with KATNAL2 acting as a downstream effector.
Teresa M. Thomas, Ching‐Yao Yang, Kexin Zhang, Liam Wetzel, Dina Bugybayeva, Julia Ferguson, Md Mahmudul Hasan, William Samsa, Nandita Patil, Anshul Dash, Tarini Gowda, Xiongwei Zhu, Feixiong Cheng, Tian Liu   +13 more
wiley   +1 more source

Proteostasis and Diseases of the Motor Unit

Frontiers in Molecular Neuroscience, 2016
The accumulation in neurons of aberrant protein species, the pathological hallmark of many neurodegenerative diseases, results from a global impairment of key cellular processes governing protein synthesis/degradation and repair mechanisms, also known as the proteostasis network (PN).
Rinaldi, C, Mäger, I, Wood, M
openaire   +3 more sources

ZER1 Restrains Pressure Overload‐Induced Cardiac Remodeling by Targeting DVL2 for Gly/N‐Degron‐Dependent Degradation

Advanced Science, EarlyView.
Pressure overload suppresses cardiomyocyte ZER1, weakening CRL2Zer1‐mediated DVL2 degradation and allowing DVL2 accumulation. Elevated DVL2 activates CaMKII‐HDAC4‐MEF2C signaling, drives fetal gene reactivation, and promotes pathological remodeling.
Mingchao Jiang, Zhehao Lin, Lu Chen, Ying Ni, Jun Zhou, Wenjuan Zhang, Huilin Wang, Peifeng Ying, Xiu Lu, Kai Wang, Qingran Kong, Naxin Xu, Dingsheng Zhao, Jianwei Li, Guohui Zhong, Xingchen Meng, Sarkawt Hamad, Junmeng Zheng, Yuan Fu, Rongjiang Qin, Xuran Chu, Shangxuan Li, Youyou Li, Yili Wu, Yi Wang, Weihong Song, Yingxian Li, Shukuan Ling   +27 more
wiley   +1 more source

Targeting the proteostasis network in Huntington's disease [PDF]

, 2019
Huntington's disease (HD) is an autosomal dominant neurodegenerative disorder caused by a polyglutamine expansion mutation in the huntingtin protein.
Pinho, BR, Oliveira, JMA, Reis, SD, Duchen, MR, Soares, TR   +4 more
core   +1 more source

Targeting defective proteostasis in the collagenopathies

, 2020
© 2019 Elsevier Ltd The collagenopathies are a diverse group of diseases caused primarily by mutations in collagen genes. The resulting disruptions in collagen biogenesis can impair development, cause cellular dysfunction, and severely impact connective ...
Wong, Madeline Y., Wong, Madeline Y, Shoulders, Matthew D., Shoulders, Matthew D   +3 more
core   +1 more source

Molecular and Cellular Hallmarks of Age‐Related Vestibular Hair Cell Degeneration

Advanced Science, EarlyView.
This study utilizes single‐cell RNA‐seq transcriptomes, advanced imaging, and electrophysiology to examine universal and cell‐type‐specific aging signatures of vestibular hair cells. The study shows that impaired hair bundle function is a key driver of age‐related vestibular dysfunction.
Samadhi Kulasooriya, Huizhan Liu, Sarath Vijayakumar, Celia Bloom, Zhenhang Xu, Shu Tu, Benjamin J. Borgmeier, Mi Zhou, Litao Tao, Bechara Kachar, David Z. He   +10 more
wiley   +1 more source

Single‐cell Transcriptome Profiling Reveals Gene Regulatory Networks and Key Genes in the Root Epidermis and Cortical Cells Associated with Early Nodulation in Glycine Max

Advanced Science, EarlyView.
Single‐cell transcriptomics of soybean roots soon after rhizobial inoculation reveals epidermal and cortical cell‐specific programs and gene‐regulatory networks acting in symbiosis establishment. We identify an ethylene‐driven regulatory circuit involving WRKY6.3/6.4 transcription factors targeting select Nod19 genes that promotes infection‐thread ...
Yongbin Zhuang, Yu Geng, Xinyu Guo, Chen Wang, Yongjing Sun, Luqi Liu, Li Liu, QianQian Gao, XinFang Zhu, Hongxiang Cao, Gengshuo Zhang, Yunxia Liu, Guobin Zhang, Baoyin Chen, Jinfei Zhang, Xin Hou, Xiaoming Li, Dajian Zhang   +17 more
wiley   +1 more source