Results 81 to 90 of about 2,946,385 (264)

PANoptosis in the pathogenesis of myelodysplastic syndromes

Molecular Oncology, EarlyView.
PANoptosis, a combination of three types of programmed cell death, is mediated by a large protein complex called a PANoptosome. In healthy bone marrow hematopoietic cells, PANoptosis is restricted by inhibitory signaling. In MDS, bone marrow cells become sensitive to the PANoptotic stimuli due to the aberrant inactivation of inhibitory signaling or ...
Rohit Thalla, Jyoti Lamichhane, Cameron Lewis, Peter Breslin, Jiwang Zhang   +4 more
wiley   +1 more source

Mechanisms of hepatocellular toxicity associated with dronedarone and other mitochondrial toxicants [PDF]

, 2014
Idiosyncratic drug-induced liver injury is a rare toxic event that typically occurs at therapeutic doses, which are generally safe to the majority of patients.
Felser, Andrea Debora
core   +1 more source

Adult-onset Sandhoff disease presenting with a motor neuron disease phenotype: clinical and mechanistic insights from patient-derived models

Acta Neuropathologica Communications
Sandhoff disease (SD) is a subtype of GM2 gangliosidosis caused by pathogenic variants in Hexosaminidase B (HEXB). It most frequently presents in infancy or early childhood, whereas adult-onset disease is rare and remains incompletely characterized. Here,
Yao Tang, Didi Shan, Hongxu Wang, Xinbo Ji, Yichang Jiao, Jianing Li, Zexin Zhan, Shuangwu Liu, Xiaohan Sun, Pengfei Lin, Jingwen Xu, Dongdong Wang, Xiulian Sun, Chuanzhu Yan, Yuying Zhao, Fuchen Liu   +15 more
doaj   +1 more source

iPSC-derived microglia: Decoding roles and therapeutic opportunities in neurodegenerative diseases

Brain Research Bulletin
Microglia originate from erythro-myeloid progenitors (EMPs) in the early embryonic yolk sac and migrate into the developing brain, where they differentiate and mature under the regulation of chemokines, cytokines, and growth factors.
Didi Shan, Hongxu Wang, Jianing Li, Yao Tang, Zexin Zhan, Chuanzhu Yan, Jingwen Xu, Fuchen Liu   +7 more
doaj   +1 more source

Efficacy of adeno-associated virus gene therapy in a MNGIE murine model enhanced by chronic exposure to nucleosides

EBioMedicine, 2020
Background: Preclinical studies have shown that gene therapy is a feasible approach to treat mitochondrial neurogastrointestinal encephalomyopathy (MNGIE).
Ferran Vila-Julià, Raquel Cabrera-Pérez, Yolanda Cámara, Miguel Molina-Berenguer, Silvia Lope-Piedrafita, Michio Hirano, Federico Mingozzi, Javier Torres-Torronteras, Ramon Martí   +8 more
doaj   +1 more source

ADP‐ribosylation: An emerging regulator of the epigenome

Molecular Oncology, EarlyView.
ADP‐ribosylation has emerged as a dynamic epigenetic signaling mechanism that modifies histones and chromatin‐associated proteins. Through coordinated PARylation and MARylation, it integrates with other histone modifications to regulate chromatin structure, transcription factor activity, and gene expression, influencing genome function and disease ...
Cristel V. Camacho, Chenqian Liu, W. Lee Kraus   +2 more
wiley   +1 more source

The emerging role of Nrf2 in mitochondrial function [PDF]

, 2015
The transcription factor NF-E2 p45-related factor 2 (Nrf2, gene name NFE2L2) allows adaptation and survival under conditions of stress by regulating the gene expression of diverse networks of cytoprotective proteins, including antioxidant, anti ...
Dinkova-Kostova, AT, Abramov, Andrey Y., Dinkova-Kostova, Albena T.; id_orcid, Abramov, AY, Dinkova-Kostova, Albena T.   +4 more
core   +1 more source

Unraveling the epigenetic code in cancer cell–tumor microenvironment crosstalk

Molecular Oncology, EarlyView.
Epigenetic regulation is a key driver of cancer development and progression. Diverse epigenetic alterations in cancer cells and components of the tumor microenvironment (TME) orchestrate their communication through multiple mechanisms. We discuss how the epigenetic code coordinates bidirectional cancer cell–TME crosstalk to promote cancer progression ...
Ji Hoon Park, Mi‐Young Kim
wiley   +1 more source

Novel POMT2 variants associated with limb-girdle muscular dystrophy R14: genetic, histological and functional studies

Orphanet Journal of Rare Diseases
Background The POMT2 gene, which encodes protein O-mannosyltransferase 2, is essential for α-dystroglycan glycosylation. Variants in POMT2 cause various disorders, including the relatively rare presentation of limb-girdle muscular dystrophy R14 (LGMDR14).
Guiguan Yang, Xiaoqing Lv, Wenjing Wu, Guangyu Wang, Mengqi Yang, Yifei Feng, Chuanzhu Yan, Meirong Liu, Pengfei Lin   +8 more
doaj   +1 more source

Arginine methylation as a regulatory ratchet in cancer: From substrate selection to malignant‐state stabilization

Molecular Oncology, EarlyView.
Arginine methylation can be viewed as a persistence‐prone post‐translational modification regulated by a network of PRMTs. Competitive and compensatory interactions among PRMTs can redistribute methylation across substrate pools shaped by sequence, structural, spatial, and environmental layers, reinforcing RNA‐processing, chromatin, and signaling ...
So Hyun Kwon, Ji Min Lee
wiley   +1 more source