Results 81 to 90 of about 45,802 (236)

The pleiotropic effects of decanoic acid treatment on mitochondrial function in fibroblasts from patients with complex I deficient Leigh syndrome [PDF]

, 2016
There is growing interest in the use of the ketogenic diet (KD) to treat inherited metabolic diseases including mitochondrial disorders. However, neither the mechanism whereby the diet may be working, nor if it could benefit all patients with ...
Bilooei, SF, Donnell, MO, Fassone, E, Heales, SJ, Hughes, SD, Kanabus, M, Rahman, S, Rutherford, T   +7 more
core   +1 more source

SETD1A Regulates Glycolysis and Senescence of Nucleus Pulposus Cells via H3K4me3–HELZ2/PPARα‐HIF1α Axis to Drive Intervertebral Disc Degeneration

Advanced Science, EarlyView.
SETD1A is a key epigenetic regulator in NPCs during IDD. In normal NPCs, it sustains H3K4me3–HELZ2/PPARα–HIF1α signaling to maintain glycolytic energy metabolism and proliferation. In degenerated NPCs, reduced SETD1A disrupts this axis, impairing glycolysis and accelerating senescence, highlighting a promising therapeutic target for IDD.
Jiawei Fu, Xue Leng, Jiang Long, Zhengao Liao, Xiaoqin An, Xuezheng Ai, Dan Long, Changqing Li, Bo Huang, Yue Zhou, Shiwu Dong, Chencheng Feng   +11 more
wiley   +1 more source

Transcriptional Regulator TonEBP Mediates Oxidative Damages in Ischemic Kidney Injury [PDF]

, 2019
TonEBP (tonicity-responsive enhancer binding protein) is a transcriptional regulator whose expression is elevated in response to various forms of stress including hyperglycemia, inflammation, and hypoxia.
Choi, Soo Youn, Kang, Hyun Je, Kwon, Hyug Moo, Kwon, Mi Jin, Lee-Kwon, Whaseon, Lim, Sun Woo, Nam, Dougu, Park, Hyun, Sanada, Satoru, Yoo, Eun Jin, Yoon, Sora   +10 more
core   +1 more source

Prmt6 Deficiency or Inhibition Restores Microglial Homeostasis and Promotes Scar‐Limited Repair in Adult Spinal Cord Injury

Advanced Science, EarlyView.
After spinal cord injury, adult microglia remain persistently activated with chronic PRMT6 (protein arginine methyltransferase 6) upregulation. Prmt6 deficiency or inhibition reestablishes microglial homeostasis and promotes a scar‐limited repairment, enhancing axonal regrowth.
Weilin Peng, Zhengqiang Wu, Yu Xiong, Zhongya Gao, Yishan Liu, Ziyi Wang, Haibin Wang, Chaofeng Han, Wenxiang Chu, Xuhua Lu   +9 more
wiley   +1 more source

The Deafness-Associated Mitochondrial DNA Mutation at Position 7445, Which Affects tRNASer(UCN) Precursor Processing, Has Long-Range Effects on NADH Dehydrogenase Subunit ND6 Gene Expression [PDF]

, 1998
The pathogenetic mechanism of the deafness-associated mitochondrial DNA (mtDNA) T7445C mutation has been investigated in several lymphoblastoid cell lines from members of a New Zealand pedigree exhibiting the mutation in homoplasmic form and from control
Attardi, Giuseppe, Enriquez, José Antonio, Fischel-Ghodsian, Nathan, Guan, Min-Xin, Lin, Catherine P., Maw, Marion A., Puranam, Ram S.   +6 more
core   +1 more source

Rewiring NADH Metabolism Through NQO1‐Mediated Redox Cycling for Targeted Follicular Lymphoma Therapy

Advanced Science, EarlyView.
ABSTRACT Follicular lymphoma (FL) remains an incurable B‐cell malignancy with high relapse rates, and conventional therapies are often limited by significant toxicity, highlighting the need for novel treatments. We identified that FL exhibits markedly low expression of NAD(P)H: quinone oxidoreductase 1 (NQO1), a key enzyme required for activating ...
Jinxing Zhang, Ziqi Wang, Ye Weng, Yulin Fu, Yuelong Jiang, Beilin Zhuang, Wenzi Zeng, Xiangchun Zhang, Bing Xu, Jie Zha, Hongjun Zhuang   +10 more
wiley   +1 more source

Pyruvate dehydrogenase complex deficiency disease is connected to regulatory loop disorder in the αV138M variant of human pyruvate dehydrogenase [PDF]

Acta Crystallographica Section A Foundations and Advances, 2018
Matthew J. Whitley, Palaniappa Arjunan, Natalia S. Nemeria, Mulchand S. Patel, Frank Jordan, William Furey   +5 more
openaire   +1 more source

Glutathione Metabolism in Renal Cell Carcinoma Progression and Implications for Therapies [PDF]

, 2019
A significantly increased level of the reactive oxygen species (ROS) scavenger glutathione (GSH) has been identified as a hallmark of renal cell carcinoma (RCC). The proposed mechanism for increased GSH levels is to counteract damaging ROS to sustain the
Meierhofer, David, Xiao, Yi
core   +2 more sources

Heat Stress and Gut Microbiome Dynamics in Poultry: Interplay, Consequences, and Mitigation Strategies

Animal Research and One Health, EarlyView.
Heat stress disrupts gut microbial balance in poultry, impairing nutrient absorption and immunity. This review outlines the interplay between thermal stress and microbiome dynamics and discusses integrative mitigation strategies, probiotics, phytogenics, cooling systems, and genetic adaptation to enhance poultry resilience.
O. E. Oke, A. I. Oni, O. A. Akosile, K. M. Oliyide, C. A. Ishola, M. O. Logunleko, O. A. Jimoh, C. P. Njoku, L. O. Fasasi, V. A. Uyanga   +9 more
wiley   +1 more source

Novel compound mutations in the mitochondrial translation elongation factor (TSFM) gene cause severe cardiomyopathy with myocardial fibro-adipose replacement [PDF]

, 2019
Primary mitochondrial dysfunction is an under-appreciated cause of cardiomyopathy, especially when cardiac symptoms are the unique or prevalent manifestation of disease.
Bonnen, Penelope E, Carbo, Miriam, Cerbelli, Bruna, D'Amati, Giulia, Della Monica, Paola Lilla, Falkous, Gavin, Giordano, Carla, Glasgow, Ruth I C, Hardy, Steven A, He, Langping, Morea, Veronica, Perli, Elena, Pignataro, Maria Gemma, Pisano, Annalinda, Re, Federica, Taylor, Robert W, Zacara, Elisabetta   +16 more
core   +2 more sources