Results 101 to 110 of about 12,014 (181)

A Nonketotic Hyperglycinemia Mouse Shows Wide‐Ranging Biochemical Consequences of Elevated Glycine, Reduced Folate One‐Carbon Charging, and Serine Deficiency

Journal of Inherited Metabolic Disease, Volume 49, Issue 1, January 2026.
ABSTRACT Nonketotic hyperglycinemia is a severe neonatal epileptic encephalopathy caused by deficient glycine cleavage enzyme activity, for which currently no effective treatment exists. Incomplete understanding of brain biochemistry represents a major knowledge gap to develop new treatments.
Michael A. Swanson, Hua Jiang, Lakshmi Divya Kolora, Rachel Molino, Richard Reisdorph, Cole R. Michel, Katrina A. Doenges, Kit‐Yi Leung, Xiangping Lin, Frank Wong, Samual Lancaster, Basil Michael, Michael Snyder, Daniella H. Hock, David A. Stroud, Tim Wood, Robert Binard, Laura Anderson‐Lehman, Uwe Christians, Erland Arning, Marisa W. Friederich, Roxanne A. Van Hove, Kenneth N. MacLean, Nicholas D. E. Greene, Johan L. K. Van Hove   +24 more
wiley   +1 more source

Mutations in mitochondrial enzyme GPT2 cause metabolic dysfunction and neurological disease with developmental and progressive features [PDF]

, 2016
Mutations that cause neurological phenotypes are highly informative with regard to mechanisms governing human brain function and disease. We report autosomal recessive mutations in the enzyme glutamate pyruvate transaminase 2 (GPT2) in large kindreds ...
Al-Saffar, Muna, Barkovich, A. James, Baytas, Ozan, Chaudhry, Haroon Rashid, Davidson, Shawn M, DeBerardinis, Ralph J., EI-Quessny, Malak, Gul Butt, Mirrat, Hill, R. Sean, Housman, David E, Imran Murtaza, Syed, Javed, Afzal, Lam, Anh-Thu N., Lizarraga, Sofia B., Mishra, Sasmita, Mochida, Ganeshwaran H., Morrow, Eric M., Nagpal, Shailender, Nakayama, Tojo, Nasir, Ramzi, Niaz, Saima, Ouyang, Qing, Partlow, Jennifer N., Rajab, Anna, Schmidt, Michael, Schwede, Matthew, Vaughan, Dylan J., Yang, Chendong, Yoo, Seung-Yun   +28 more
core   +1 more source

Magnetic Resonance Spectroscopy of Cystathionine and 2‐Hydroxyglutarate in Brain Tumors

NMR in Biomedicine, Volume 39, Issue 1, January 2026.
Cystathionine and 2‐hydroxyglutarate were evaluated in 38 glioma patients with TE 97ms PRESS at 3T. Cystathionine level was significantly higher in IDH‐mutant 1p/19q‐codeleted gliomas than in IDH‐mutant non‐codeleted gliomas (p = 0.002). The sensitivity and specificity of the cystathionine measures with respect to 1p/19q status were 0.8 and 0.79, while
Changho Choi, Mai Huynh, Zoltan Kovacs, William J. Behof, Wellington Pham, Sandeep K. Ganji, Zhongxu An, Toral R. Patel, Elizabeth A. Maher, Julia D. Berry, Bret C. Mobley, Larry T. Davis, Colin D. McKnight, Sumit Pruthi, Ryan T. Merrell, Alexander C. Mohler, Patrick D. Kelly, Reid C. Thompson   +17 more
wiley   +1 more source

Non Communicable Diseases and Infections Refuse to go away Despite Current Knowledge and Scientific Advances. [PDF]

, 2014
No abstract ...
Tumwine, James K
core   +2 more sources

NRF2 signalling in cytoprotection and metabolism

British Journal of Pharmacology, Volume 183, Issue 1, Page 101-114, January 2026.
The KEAP1‐NRF2 system plays a central role in cytoprotection in defence mechanisms against oxidative stress. The KEAP1‐NRF2 system has been regarded as a sulfur‐utilizing cytoprotective mechanism, because KEAP1 serves as a biosensor for electrophiles by using its reactive thiols and NRF2 is a transcriptional factor regulating genes involved in sulfur ...
Shohei Murakami, Yusuke Kusano, Keito Okazaki, Takaaki Akaike, Hozumi Motohashi   +4 more
wiley   +1 more source

Release of GLP-1 and PYY in response to the activation of G protein-coupled bile acid receptor TGR5 is mediated by Epac/PLC-ε pathway and modulated by endogenous H2S [PDF]

, 2014
Activation of plasma membrane TGR5 receptors in enteroendocrine cells by bile acids is known to regulate gastrointestinal secretion and motility and glucose homeostasis.
Ancy D. Nalli, Arun J. Sanyal, Divya P. Kumar, John R. Grider, Karnam S. Murthy, Senthilkumar Rajagopal, Sunila Mahavadi, Vanitha Bala   +7 more
core   +2 more sources

Supersulfide biology and translational medicine for disease control

British Journal of Pharmacology, Volume 183, Issue 1, Page 115-130, January 2026.
Abstract For decades, the major focus of redox biology has been oxygen, the most abundant element on Earth. Molecular oxygen functions as the final electron acceptor in the mitochondrial respiratory chain, contributing to energy production in aerobic organisms. In addition, oxygen‐derived reactive oxygen species including hydrogen peroxide and nitrogen
Uladzimir Barayeu, Tomohiro Sawa, Motohiro Nishida, Fan‐Yan Wei, Hozumi Motohashi, Takaaki Akaike   +5 more
wiley   +1 more source

Genetically encoded fluorescent redox probes. [PDF]

, 2013
Redox processes are involved in almost every cell of the body as a consequence of aerobic life. In the past decades, redox biology has been increasingly recognized as one of the key themes in cell signaling.
Ai, Hui-Wang, Ren, Wei
core   +3 more sources

Biosynthesis of H2S and Siderophores Targeting Gram‐Negative Bacterial Resistance to Reactive Oxygen Species

Advanced Science, Volume 12, Issue 45, December 4, 2025.
Discover how Gram‐negative bacteria develop resistance to reactive oxygen species, and how scientists aim to overcome this challenge. Abstract Reactive oxygen species (ROS) are a promising alternative bactericide. However, it is questioned that bacteria can potentially develop resistance to ROS, similar to their resistance against antibiotics and ...
Congyang Mao, Wanyu Jin, Yiming Xiang, Yizhou Zhu, Jun Wu, Xiangmei Liu, Shuilin Wu, Wei Qiao, Kenneth M. C. Cheung, Kelvin Wai Kwok Yeung   +9 more
wiley   +1 more source

Impact of hyperglycemia on cystathionine-γ-lyase expression during resuscitated murine septic shock

Intensive Care Medicine Experimental, 2017
Background Cystathionine-γ-lyase (CSE) was shown to have a regulatory role in glucose metabolism. Circulatory shock can induce metabolic stress, thereby leading to hyperglycemia and mitochondrial dysfunction.
Tamara Merz, Josef A. Vogt, Ulrich Wachter, Enrico Calzia, Csaba Szabo, Rui Wang, Peter Radermacher, Oscar McCook   +7 more
doaj   +1 more source