Results 71 to 80 of about 47,907 (257)

Pyruvate dehydrogenase deficiency in a boy: a clinical case

Journal of Clinical Practice
Pyruvate dehydrogenase deficit is one of the forms of severe hereditary mitochondrial metabolic diseases characterized by impaired energy metabolism and manifested by a wide range of neurological symptoms.
Galina Yuryevna Poretskova, Elena Anatolievna Kalinina, Natalia Nikolaevna Korotkova, Olga Gennadievna Bolgarova, Natalia Nikitichna Kuznecova, Shamil Ildarovich Gaisin, Elizabeth Olegovna Beschastnaya   +6 more
semanticscholar   +1 more source

Connexin43 Deficiency Leads to Ventricular Arrhythmias by Reprogramming Proline Metabolism

Advanced Science, EarlyView.
The study demonstrated that connexin43 (Cx43) knockout caused arrhythmic phenotype and decreased proline content in vitro and in vivo. Mechanistically, Cx43 interacts with the amino acid transporter SNAT2 (sodium‐dependent neutral amino acid transporter), and its deficiency disrupts proline transport and metabolism.
Hangying Ying, Hangping Fan, Yunhe Wang, Ruhong Jiang, Dongsheng Cai, Hui Cheng, Hao Wang, Chenyang Jiang, Ping Liang   +8 more
wiley   +1 more source

Biosynthesis of Kaitocephalin: A Neuroprotective Natural Product Featuring a Peptide‐Like yet Nonpeptidic Scaffold

Angewandte Chemie, EarlyView.
Kaitocephalin (KCP) is a neuroprotective fungal metabolite with a unique scaffold of amino acids linked via C–C bonds. Genome‐transcriptome analyses identified its biosynthetic gene cluster (kpb cluster) in Eupenicillium shearii. LC‐MS/MS profiling identified four new KCP‐related compounds.
Yukari Maeno, Taro Shiraishi, Naoya Saito, Jun‐ichi Maruyama, Kazuo Shin‐ya, Tomohisa Kuzuyama   +5 more
wiley   +2 more sources

Fibroblast-Like Synoviocytes Glucose Metabolism as a Therapeutic Target in Rheumatoid Arthritis. [PDF]

, 2019
Metabolomic studies show that rheumatoid arthritis (RA) is associated with metabolic disruption that may be therapeutically targetable. Among them, glucose metabolism and glycolytic intermediaries seem to have an important role in fibroblast-like ...
de Oliveira, Patricia Gnieslaw, Farinon, Mirian, Guma, Monica, Miyamoto, Shigeki, Sanchez-Lopez, Elsa   +4 more
core   +1 more source

HIBCH mutations can cause Leigh-like disease with combined deficiency of multiple mitochondrial respiratory chain enzymes and pyruvate dehydrogenase

Orphanet Journal of Rare Diseases, 2013
BackgroundDeficiency of 3-hydroxy-isobutyryl-CoA hydrolase (HIBCH) caused by HIBCH mutations is a rare cerebral organic aciduria caused by disturbance of valine catabolism.
S. Ferdinandusse, H. Waterham, S. Heales, Garry K. Brown, I. Hargreaves, J. Taanman, R. Gunny, L. Abulhoul, R. Wanders, P. Clayton, J. Leonard, S. Rahman   +11 more
semanticscholar   +1 more source

SLC2A3‐Mediated Lactate Metabolism Promotes Lung Cancer Bone Metastasis by Modulating P53 Lactylation and Immune Evasion

Advanced Science, EarlyView.
SLC2A3 derived lactate promotes metastasis through p53 lactylation at K120 and osteoclast differentiation. Pharmacological inhibition of SLC2A3 upregulates PD‐1 expression on CD8+ T cells via lactate induced p53 lactylation, which modulates immune evasion. ABSTRACT Bone metastasis is a devastating consequence of lung cancer.
Yi Ding, Yuying Tian, Wenjie Ren, Xianglin Hu, Mengjuan Li, Bei Liu, Chen chen, Yunhan Lu, Lei Li, Wangjun Yan, Kun Li   +10 more
wiley   +1 more source

A review of the molecular mechanisms underlying the development and progression of cardiac remodeling [PDF]

, 2017
Pathological molecular mechanisms involved in myocardial remodeling contribute to alter the existing structure of the heart, leading to cardiac dysfunction.
Angelini, Francesco, Bordin, Antonella, Carrizzo, Albino, Chimenti, Isotta, DE FALCO, Elena, Forte, Maurizio, Frati, Giacomo, Nocella, Cristina, Pagano, Francesca, Palmerio, Silvia, Schiavon, Sonia, Schirone, Leonardo, Sciarretta, Sebastiano, Valenti, Valentina, Vecchione, Carmine, Yee, Derek   +15 more
core   +3 more sources

Differential inhibition of PDKs by phenylbutyrate and enhancement of pyruvate dehydrogenase complex activity by combination with dichloroacetate

Journal of Inherited Metabolic Disease, 2015
Pyruvate dehydrogenase complex (PDHC) is a key enzyme in metabolism linking glycolysis to tricarboxylic acid cycle and its activity is tightly regulated by phosphorylation catalyzed by four pyruvate dehydrogenase kinase (PDK) isoforms.
R. Ferriero, Clara Iannuzzi, Giuseppe Manco, N. Brunetti‐Pierri   +3 more
semanticscholar   +1 more source

Oncolytic Probiotics with Molecular Pili for Solid Tumor Therapy

Advanced Science, EarlyView.
This study identifies Lactobacillus rhamnosus as an intrinsic oncolytic agent that triggers tumor metabolic collapse via calcium‐dependent ROS bursts. By chemically anchoring collagen‐targeting “molecular pili” to the bacterial surface, the engineered non‐transgenic probiotic (LR@MP) achieves targeted colonization and potent solid tumor suppression ...
Haodong Ge, Chengsheng Ding, Xiao Yang, Si Gao, Changjie Yang, Yuchen Hou, Hongye Wang, Linke Bian, Hao Zhong, Yifan Qu, Luyang Zhang, Junjun Ma, Zhengwei Cai, Wenguo Cui, Minhua Zheng   +14 more
wiley   +1 more source

Metabolic Regulation of Epithelial to Mesenchymal Transition: Implications for Endocrine Cancer [PDF]

, 2019
York University ...
Bhattacharya, Debasmita, Scimè, Anthony
core   +1 more source