Results 41 to 50 of about 22,061 (226)

Integration of Spatiotemporal Multi‐Omics in Peach Fruit Unravels a Metabolic Niche and the Genetic Basis of Trichome‐Mediated Stress Adaptation

Advanced Science, EarlyView.
This study constructed the first spatiotemporal multi‐omics map of peach fruit and discovered a key candidate gene that synergistically regulates trichome development and drought tolerance through the jasmonic acid signaling pathway, providing insights into the coupling mechanism between development and stress resistance.
Zhixin Liu, Ke Cao, Liping Guan, Aizhi Qin, Yong Li, Zihao Zhao, Yinpeng Zhang, Yaping Zhou, Lirong Wang, Xuwu Sun   +9 more
wiley   +1 more source

Pyruvate Dehydrogenase Phosphatase Deficiency: Identification of the First Mutation in Two Brothers and Restoration of Activity by Protein Complementation

, 2005
Context: Pyruvate dehydrogenase phosphatase (PDP) deficiency has been previously reported as an enzymopathy, but the genetic basis for such a defect has never been established.
Baumgartner, E Regula, Robinson, Brian H, Levandovskiy, Valeriy, MacKay, Neviana, Baumgartner, Matthias R; https://orcid.org/, Maj, Mary C, Cameron, Jessie M, Addis, Jane   +7 more
core   +1 more source

Carnitine : analytical and physiological aspects [PDF]

, 2012
Carnitine is an endogenous amino acid derivative which plays a key role in energy metabolism. Through acylation of its ß-hydroxy group, carnitine is involved in the interorganellar transport of long-chain fatty acids and in the regulation of the coenzyme
Bourqui, Réjane Morand
core   +1 more source

Disruption of Treg Homeostasis in Rheumatoid Arthritis via Ferroptosis‐Mediated ETC Collapse and TXK‐STAT3/PLCγ1 Activation

Advanced Science, EarlyView.
In rheumatoid arthritis, synovial Tregs accumulate but are functionally impaired due to iron overload‐induced ferroptosis. This triggers mitochondrial dysfunction and TXK tyrosine kinase‐mediated signaling, leading to Treg destabilization and inflammation.
Jingrong Chen, Xiao Guan, Kexu Xiong, Xiaoyi Shi, Luyao Wu, Chuanxin Su, Jun Zhao, Rongzhen Liang, Huimin Wang, Junlong Dang, Ye Chen, Yiding Xiong, Chi Zhou, Futao Zhao, Zhenhang Zhu, Xiaoli Fan, Li Zhou, Nancy Olsen, Yutong Jiang, Song Guo Zheng   +19 more
wiley   +1 more source

Personalized medicine – parenteral ketogenic diet in pyruvate dehydrogenase deficiency in the ICU; what do we need to know about it?

, 2022
Manjak kompleksa piruvat dehidrogenaze (engl. Pyruvate Dehydrogenase Complex Deficiency, PDCD) rijedak je genetski neurometabolički poremećaj. Pripada u skupinu mitohondrijskih bolesti.
Carev, Mladen, Stojanović Stipić, Sanda, Frankić, Mileva, Kljaković Gašpić, Toni   +3 more
core   +1 more source

Lymphoid‐Tissue‐on‐Chip Recapitulates Human Antibody Responses In Vitro

Advanced Science, EarlyView.
The presented lymphoid‐tissue‐on‐chip system allows culture of primary human tonsil cells at organotypic high density under perfusion for up to 4 weeks, emulates immune response to soluble vaccines and vaccination via peripheral antigen‐presenting cells and represents a useful tool to assess cellular interactions during homeostasis, immune responses ...
Claudia Teufel, Anna‐Sophie Schlemmer, Andrea W. Eiken, Zachary W. Wagoner, Dennis Vöhringer, Lena Christ, Alex Dulovic, Patrick Marsall, Madeleine Fandrich, Eduardo J. S. Brás, Julia Marzi, Friederike Bärhold, Julia Philipp, Sven Becker, Lisa E. Wagar, Peter Loskill   +15 more
wiley   +1 more source

Mutations in human lipoyltransferase gene LIPT1 cause a Leigh disease with secondary deficiency for pyruvate and alpha-ketoglutarate dehydrogenase.

, 2013
International audienceBACKGROUND: Synthesis and apoenzyme attachment of lipoic acid have emerged as a new complex metabolic pathway. Mutations in several genes involved in the lipoic acid de novo pathway have recently been described, but no mutation was ...
Hamel, Yamina, Christine Barnerias, Florence Habarou, Agnès Delahodde, de Lonlay, Pascale, Nathalie Boddaert, Hélène Delpech, Patrick Nitschke, Yamina Hamel, Claude Sardet, Ottolenghi, Chris, Dominique Chrétien, Yohan Soreze, Delahodde, Agnès, Pascale de Lonlay, Bole-Feysot, Christine, Delpech, Hélène, Soreze, Yohan, Mamoune, Asmaa, Chrétien, Dominique, Boddaert, Nathalie, Hubert, Laurence, Asmaa Mamoune, Christine Bole-Feysot, Isabelle Correia, Audrey Boutron, Sardet, Claude, Nonnenmacher, Luc, Habarou, Florence, Laurence Hubert, Luc Nonnenmacher, Boutron, Audrey, Nitschke, Patrick, Chris Ottolenghi, Barnerias, Christine, Correia, Isabelle   +35 more
core   +1 more source

Cuproptosis and Mitophagy Mediated by the THUMPD1/IGF2R‐Dependent Suppression of AKT and Activation of AMPK Signaling Suppress Lung Adenocarcinoma Progression

Advanced Science, EarlyView.
THUMPD1 drives a tumor‐suppressive signaling cascade in lung adenocarcinoma by promoting IGF2R expression. IGF2R associates with PPP2R1A to suppress AKT and activate AMPK, leading to SLC31A1 upregulation and copper accumulation. Elevated copper disrupts mitochondrial metabolism and induces excessive mitophagy, thereby restraining tumor growth and ...
Kai Wu, Bo Qin, Zhuoyu Gu, Weizheng Ding, Xiaoming Chen, Kaishang Zhang, Yujin Qiao, Shuang Yuan, Song Zhao, Xiangnan Li, Peng Zhang   +10 more
wiley   +1 more source

Metabolic Imbalance Triggers Adaptive Remodeling to Accelerate Diploidization in Murine Haploid Embryonic Stem Cells

Advanced Science, EarlyView.
In this article, Shuai and colleagues demonstrate that metabolic remodeling drives self‐diploidization in murine haploid ESCs (haESCs). Mitochondrial dysfunction and imbalanced pyruvate metabolism underlie this process. Genome‐wide screening using haESCs identifies key mitochondrial quality‐control related genes, enabling a metabolism‐based medium that
Yi Fu, Wenhao Zhang, Yifan Zhang, Yu He, Yi Du, Yiding Zhao, Chunmeng Yao, Shengyi Sun, Xiaoyan Sheng, Qian Gao, Chao Tong, Ling Shuai   +11 more
wiley   +1 more source

Energy substrate metabolism in pyruvate dehydrogenase complex deficiency

, 2014
Pyruvate dehydrogenase (PDH) deficiency is an inherited disorder of carbohydrate metabolism, resulting in lactic acidosis and neurological dysfunction. In order to provide energy for the brain, a ketogenic diet has been tried.
Stenlid, Maria Halldin,, von Döbeln, Ulrika,, von Döbeln, Ulrika, Ahlsson, Fredrik,, Forslund, Anders H,, Gustafsson, Jan,   +5 more
core   +1 more source