Results 51 to 60 of about 155,517 (152)

Screening of a clinically and biochemically diagnosed SOD patient using exome sequencing: A case report with a mutations/variations analysis approach

Egyptian Journal of Medical Human Genetics, 2016
Background: Sulfite oxidase deficiency (SOD) is a rare neurometabolic inherited disorder causing severe delay in developmental stages and premature death.
Mohamad-Reza Aghanoori, Ghazaleh Mohammadzadeh Shahriary, Mahdi Safarpour, Ahmad Ebrahimi   +3 more
doaj   +1 more source

Ceruloplasmin, copper, selenium, iron, zinc, and manganese levels in normal and sulfite oxidase deficient rat plasma: Effects of sulfite exposure

, 2008
A noticeable effect of sulfite treatment was observed on the plasma ceruloplasmin ferroxidase activity of rats with normal sulfite oxidase activity when compared to normal controls. The plasma levels of selenium, iron, and zinc were unaffected by sulfite
Vural, H., Herken, Emine Nur, Küçükatay, Vural, Kucukatay, M.B., Kocamaz, Erdoğan, Demirin, H., Demirin, Hilmi, Kucukatay, Melek Bor, Vural, Huseyin   +8 more
core   +1 more source

One Hundred Years of Sulfido Ligand in the Molybdenum Cofactor: New Perspectives

ChemBioChem, Volume 27, Issue 19, 14 October 2026.
The terminal sulfido ligand in mononuclear molybdoenzymes has been appreciated for almost 125 years, with the characterization of Schardinger's enzyme in cow's milk, now known to be xanthine oxidase, that was isolated in 1902 in Vienna, Austria. The ligand is found in molybdoenzymes identified across all kingdoms of life.
Silke Leimkühler, Benjamin R. Duffus, Carola Schulzke   +2 more
wiley   +1 more source

The Human Mercaptopyruvate Sulfurtransferase TUM1 Is Involved in Moco Biosynthesis, Cytosolic tRNA Thiolation and Cellular Bioenergetics in Human Embryonic Kidney Cells

Biomolecules, 2023
Sulfur is an important element that is incorporated into many biomolecules in humans. The incorporation and transfer of sulfur into biomolecules is, however, facilitated by a series of different sulfurtransferases.
Moses Olalekan Ogunkola, Gaelle Guiraudie-Capraz, Francois Feron, Silke Leimkühler   +3 more
doaj   +1 more source

Recent Progress in Understanding the Mechanism of Formate Dehydrogenases

ChemBioChem, Volume 27, Issue 19, 14 October 2026.
A hydride transfer mechanism for the molybdenum‐ and tungsten‐containing formate dehydrogenases. Recent work concerning the reaction mechanism of the molybdenum‐containing formate dehydrogenases is discussed, focusing on two alternate mechanisms of action for the enzyme involving either formation of CO2 or bicarbonate as the immediate product of the ...
Russ Hille, Dimitri Niks
wiley   +1 more source

Case Report: Compound Heterozygous Variants in MOCS3 Identified in a Chinese Infant With Molybdenum Cofactor Deficiency

Frontiers in Genetics, 2021
Background: The molybdenum cofactor (Moco) deficiency in humans results in the inactivity of molybdenum-dependent enzymes and is caused by pathogenic variants in MOCS1 (Molybdenum cofactor synthesis 1), MOCS2 (Molybdenum cofactor synthesis 2), and GPHN ...
Qi Tian, Yang Cao, Li Shu, Li Shu, Li Shu, Yongjun Chen, Ying Peng, Yaqin Wang, Yuanyuan Chen, Hua Wang, Hua Wang, Xiao Mao, Xiao Mao   +12 more
doaj   +1 more source

Functional deficiencies of sulfite oxidase: Differential diagnoses in neonates presenting with intractable seizures and cystic encephalomalacia

, 2010
Sulfite oxidase is a mitochondrial enzyme encoded by the SUOX gene and essential for the detoxification of sulfite which results mainly from the catabolism of sulfur-containing amino acids. Decreased activity of this enzyme can either be due to mutations
GUNDUZ, Aysegul, Dantas Pinto, Kylvia Giselle, Rodrigues Funayama, Carolina Araujo, PINTO, Kylvia Giselle Dantas, Araujo Rodrigues Funayama, Carolina, Dos Santos, Leticia Yanasse, TASKIRAN, Emine, CAMELO JUNIOR, Jose Simon, TUYSUZ, Beyhan, LAM, Ching-Wan, SANTOS, Leticia Yanasse Dos, REISS, Jochen, FUNAYAMA, Carolina Araujo Rodrigues, TURCATO, Marlene de Fatima, Yanasse Dos Santos, Letícia, Sass, Jörn Oliver, KORKMAZ, Baris, SASS, Joern Oliver, YALCINKAYA, Cengiz, WALTER, Melanie, Fátima Turcato, Marlene de, De Fátima Turcato, Marlene, Saß, Jörn-Oliver, Baris Korkmaz, Beyhan Tuysuz, Yalcinkaya, C, José Simon Camelo Junior, Taskiran, E, Lam, CW, Aysegul Gunduz, Dantas Pinto, KG, Walter, M, Carolina Araujo Rodrigues Funayama, Cengiz Yalcinkaya, Marlene de Fátima Turcato, Jörn Oliver Sass, Gunduz, A, Melanie Walter, de Fátima Turcato, M, Ching-Wan Lam, Kylvia Giselle Dantas Pinto, Jochen Reiss, Sass, JO, Letícia Yanasse Dos Santos, Korkmaz, B, Araujo Rodrigues Funayama, C, Reiss, J, Camelo Junior, JS, Emine Taskiran, Tuysuz, B, Yanasse Dos Santos, L   +50 more
core   +1 more source

Anti‐Browning and Prebiotic Effects of White Tea Extract on Fresh‐Cut Lotus Root

Food Science &Nutrition, Volume 14, Issue 10, October 2026.
PPO‐driven browning limits the shelf life of fresh‐cut lotus root. Lotus root PPO was identified and its 3D structure modeled, with catechin and caffeic acid as specific substrates binding via 8 and 5 hydrogen bonds, respectively. White tea extract inhibited PPO and delayed browning of lotus root slices. White tea‐treated lotus root exhibited prebiotic
Jingfang Li, Chuangkai Xing, Fan Jiang, Xiaoru Liu   +3 more
wiley   +1 more source

Molybdenum metabolism in plants and crosstalk to iron

Frontiers in Plant Science, 2014
In the form of molybdate the transition metal molybdenum is essential for plants as it is required by a number of enzymes that catalyze key reactions in nitrogen assimilation, purine degradation, phytohormone synthesis, and sulfite detoxification ...
Florian eBittner
doaj   +1 more source

Effect of sulfite on red blood cell deformability ex vivo and in normal and sulfite oxidase-deficient rats in vivo

, 2005
The effect of sulfite, a widely used food additive, on red blood cell deformability ex vivo and in vivo was investigated. Ex vivo experiments were conducted in human blood exposed to sulfite (5, 10 and 20 mM).
Melek Bor-Kucukatay, Vural Kucukatay, Baskurt, O.K., Küçükatay, Vural, Ağar, Aysel, Oguz Kerim Baskurt, Bor-Küçükatay, Melek, Aysel Agar   +7 more
core   +1 more source