Results 81 to 90 of about 66,093 (301)

The Mitochondrial Metallochaperone SCO1 Is Required to Sustain Expression of the High-Affinity Copper Transporter CTR1 and Preserve Copper Homeostasis

Cell Reports, 2015
Human SCO1 fulfills essential roles in cytochrome c oxidase (COX) assembly and the regulation of copper (Cu) homeostasis, yet it remains unclear why pathogenic mutations in this gene cause such clinically heterogeneous forms of disease.
Christopher J. Hlynialuk, Binbing Ling, Zakery N. Baker, Paul A. Cobine, Lisa D. Yu, Aren Boulet, Timothy Wai, Amzad Hossain, Amr M. El Zawily, Pamela J. McFie, Scot J. Stone, Francisca Diaz, Carlos T. Moraes, Deepa Viswanathan, Michael J. Petris, Scot C. Leary   +15 more
doaj   +1 more source

The role of Coenzyme Q10 in statin-associated myopathy [PDF]

, 2009
Statins, or 3-hydroxyl-3-methylglutaryl coenzyme HMG-CoA reductase inhibitors,\ud are cholesterol-lowering drugs which are frequently used in the primary and secondary\ud prevention of coronary artery disease. Current research and recommendations support\
Kalra, Dr Sanjay
core  

Multi‐Omics Insights Into the Mechanisms of Early Muscle Fiber Difference and Transformation Between Lean‐Type and Chinese Indigenous Pigs

Advanced Science, EarlyView.
Multi‐omics analyses uncover breed‐specific cis‐regulatory landscapes and higher‐order chromatin architectural differences that underlie early postnatal muscle fiber divergence in pigs. A super‐enhancer upstream of PPP3CB recruits MEF2C to activate PPP3CB transcription, while the PPP3CB–MEF2C positive feedback loop promotes oxidative muscle fiber ...
Shuailong Zheng, Hainan Wu, Min Liu, Kunpeng Wu, Shuntao Huang, Zulfiqar Ahmed, Lenox Pius, Mengjin Zhu, Dequan Xu   +8 more
wiley   +1 more source

Copper Depletion Nanoparticles Potentiate Cancer Immunotherapy by Avoiding Innate and Adaptive Immune Resistance

Advanced Science, EarlyView.
A mitochondria‐targeted copper depletion nanoplatform (CYN‐CDA@Alb) was developed to selectively disrupt tumor mitochondria copper, which then reprogrammed the tumor immune microenvironment by depressing PD‐L1 and CD47 expression simultaneously. By doing this, CYN‐CDA@Alb reversed radiotherapy‐induced immune tolerance, showing the potential usage of ...
Zaigang Zhou, Ke Li, Xuelan Li, Lei Yi, Zhengxiang Wang, Huan Ding, Sheng Wu, Feiyu Liu, Yuan Li, Rui Cheng, Jianliang Shen   +10 more
wiley   +1 more source

Schizophrenia and Leigh syndrome, a simple comorbidity or the same etiopathogeny: about a case

The Pan African Medical Journal, 2015
Leigh syndrome is a mitochondrial encephalomyopathy that occurs due to "cytochrome c oxidase deficiency". Few psychiatric disorders have been defined that are associated with Leigh syndrome.
Leila Mnif1, Rim Sellami, Jawaher Masmoudi   +1 more
doaj   +1 more source

When should MERRF (myoclonus epilepsy associated with ragged-red fibers) be the diagnosis?

Arquivos de Neuro-Psiquiatria, 2014
Myoclonic epilepsy associated with ragged red fibers (MERRF) is a rare mitochondrial disorder. Diagnostic criteria for MERRF include typical manifestations of the disease: myoclonus, generalized epilepsy, cerebellar ataxia and ragged red fibers (RRF) on ...
Paulo José Lorenzoni, Rosana Herminia Scola, Cláudia Suemi Kamoi Kay, Carlos Eduardo S. Silvado, Lineu Cesar Werneck   +4 more
doaj   +1 more source

Isolation and Characterization of Glycerol-3-Phosphate Dehydrogenase-Defective Mutants of \u3cem\u3eNeurospora crassa\u3c/em\u3e [PDF]

, 1978
Three glycerol-nonutilizing mutants deficient in the mitochondrial glycerol-3-phosphate (G3P) dehydrogenase (EC 1.1.99.5) were isolated from inlts derivatives of Neurospora crassa following inositolless death at elevated temperatures on minimal glycerol ...
Courtright, James B., Denor, Patrick F.
core   +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

m.10010T>C Mitochondrial Disease: A Case Report With Hypoparathyroidism and Review of the Literature

American Journal of Medical Genetics Part A, EarlyView.
ABSTRACT Mitochondria are essential intracellular organelles that play a critical role in cellular metabolism, including the regulation of intracellular calcium signaling. Advances in genomic sequencing have facilitated the identification of rare pathogenic mitochondrial DNA (mtDNA) genetic variants in patients with unexplained endocrine disorders.
Jacob Mohr, Anja Lisbeth Frederiksen, Morten Duno, Anne Pernille Hermann, Trine Maxel Juul, Simone Rask Nielsen   +5 more
wiley   +1 more source

Characterization of Shy1, the Schizosaccharomyces pombe homolog of human SURF1

Scientific Reports
Cytochrome c oxidase (complex IV) is the terminal enzyme in the mitochondrial respiratory chain. As a rare neurometabolic disorder caused by mutations in the human complex IV assembly factor SURF1, Leigh Syndrome (LS) is associated with complex IV ...
Ying Luo, Yuanqi Xu, Fawad Ahmad, Gang Feng, Ying Huang   +4 more
doaj   +1 more source