Results 61 to 70 of about 532,713 (215)

Clinical, Biochemical, and Molecular Studies and Treatment of Pyridoxine-Dependent Epilepsy

Pediatric Neurology Briefs, 2013
Researchers at Autonomous University of Madrid, and other centers in Spain studied the clinical, biochemical, and genetic spectrum of pyridoxine-dependent epilepsy (PDE) in 12 patients with the clinically proven diagnosis.
J. Gordon Millichap
doaj   +1 more source

Integrated Analysis of Molybdenum Nutrition and Nitrate Metabolism in Strawberry

Frontiers in Plant Science, 2020
Molybdenum (Mo) is a component of the Mo cofactor (Moco) of nitrate reductase (NR) and is therefore essential for nitrate metabolism. However, little is known about Mo deficiency phenotypes or about how physiological and molecular mechanisms of Mo uptake
Li Liu, Hongmei Shi, Shaoxuan Li, Mingyue Sun, Rui Zhang, Yongmei Wang, Fengshan Ren   +6 more
doaj   +1 more source

Zinc Fingers as Programmable Metalloprotein Scaffolds for Altering Pathogenic Biomolecular Phase Transitions

Advanced Science, EarlyView.
Modular zinc finger (ZF) metalloproteins function as programmable biomolecular scaffolds that modulate pathogenic amyloid assembly. Through direct protein–protein interactions, ZF arrays alter amyloid phase behavior and redirect β‐sheet fibrillization toward nontoxic aggregates.
Seongmin Park, Yunha Hwang, Jimin Kwak, Yuxi Lin, Hye‐Yeon Park, Eunju Nam, Jiyeon Han, Soyeon Park, Hyunyong Kim, Ju‐Eun Kim, Hyun Goo Kang, Jiyong Park, Young‐Ho Lee, Kyoung‐Shim Kim, Chul‐Ho Lee, Seung Jae Lee, Mi Hee Lim   +16 more
wiley   +1 more source

Continuous Molecular Monitoring Using Electrochemical Aptamer‐Based Sensors: Remaining Challenges for Long‐Term In Vivo Deployment

Advanced Science, EarlyView.
Continuous molecular monitoring using electrochemical aptamer‐based sensors enables real‐time tracking of dynamic biomarkers with transformative potential for precision medicine. Achieving clinically relevant month‐long stability remains a central challenge due to multiple degradation pathways.
Shibo Liu, Xiangling Li, Wei Ouyang
wiley   +1 more source

Proteins Structure Models in the Evaluation of Novel Variant (C.472_477del) in the MOCS2 Gene

Diagnostics, 2020
(1) Background: Molybdenum cofactor deficiency type B (MOCODB, #252160) is a rare autosomal recessive metabolic disorder characterized by intractable seizures of neonatal-onset, muscular spasticity, accompanying with hypouricemia, elevated urinary ...
Aleksandra Jezela-Stanek, Witold Blaz, Artur Gora, Malgorzata Bochenska, Katarzyna Kusmierska, Jolanta Sykut-Cegielska   +5 more
doaj   +1 more source

A case of molybdenum cofactor deficiency

Sri Lanka Journal of Child Health, 2017
Sri Lanka Journal of Child Health, 2017; 46(3): 272 ...
Dinesha Maduri Vidanapathirana, Subashinie Jayasena, Eresha Anandanie Jasinge, Pyara Ratnayake, Lynette Fairbanks   +4 more
openaire   +2 more sources

Bioenergetic Materials for Tissue Regeneration: Modulating Metabolism to Promote Cellular Anabolism

Advanced Science, EarlyView.
Bioenergetic materials (BEMs) modulate cellular metabolism to promote tissue regeneration. Their principal mechanisms of action include metabolic substrate supplementation, metabolic enzyme modulation, organelle transfer, electrical current regulation, redox homeostasis modulation, and oxygen tension modulation.
Yuchen He, Yuwen Wang, Jonathan F. Gong, Yiting Lei, Fei Jin, Weihong Zhu, Rocky S. Tuan, Zhong Alan Li   +7 more
wiley   +1 more source

Molybdenum cofactor deficiency: Review of 12 cases (MoCD and review)

, 2013
Molybdenum cofactor deficiency is a rare inborn error of metabolism. The major clinical symptoms are intractable neonatal seizures, progressive encephalopathy, facial dysmorphic features and feeding difficulties.
YİŞ, ULUÇ, Topcu, Yasemin, Ichida, Kimiyoshi, Bayram, Erhan, Kurul, Semra Hiz, Cakmakci, HANDAN, Karakaya, Pakize   +6 more
core   +1 more source

Structural and functional insights into the catalytic inactivity of the major fraction of buffalo milk xanthine oxidoreductase. [PDF]

PLoS ONE, 2014
BACKGROUND: Xanthine oxidoreductase (XOR) existing in two interconvertible forms, xanthine dehydrogenase (XDH) and xanthine oxidase (XO), catabolises xanthine to uric acid that is further broken down to antioxidative agent allantoin.
Kaustubh S Gadave, Santanu Panda, Surender Singh, Shalini Kalra, Dhruba Malakar, Ashok K Mohanty, Jai K Kaushik   +6 more
doaj   +1 more source

Supramolecular Chiral Assemblies from Benzoselenadiazole‐Alanine‐Acylhydrazone Conjugates Enable Cardioprotection

Advanced Science, EarlyView.
The C3‐symmetric conjugate TASe, which integrates benzoselenadiazole, alanine, and acylhydrazone modules, self‐assembles into a chalcogen‐/hydrogen‐bonded porous framework in the solid state and uniform chiral nanospheres in aqueous media. These assemblies protect cardiomyocytes from doxorubicin‐induced oxidative injury by scavenging intracellular ...
Jinkui Teng, Renjing Yang, Yu Du, Jingzhong Duan, Yiming Xu, Zhihua He, Guanghui Wang, Wenjing Tian, Aijie Liu, Haifeng Chen, Yun‐Bao Jiang, Xiaosheng Yan   +11 more
wiley   +1 more source