Results 41 to 50 of about 532,713 (215)

The History of the Molybdenum Cofactor—A Personal View

open access: yesMolecules, 2022
The transition element molybdenum (Mo) is an essential micronutrient for plants, animals, and microorganisms, where it forms part of the active center of Mo enzymes.
Ralf R. Mendel
doaj   +1 more source

Microelements and Inherited Metabolic Diseases

open access: yesActa Medica, 2002
In addition to the main groups of inherited metabolic diseases, including mitochondrial, peroxisomal and lysosomal defects, organic acidurias, porphyrias, defects of amino acids, saccharides and fatty acids metabolism, disorders of transport and ...
Eliška Marklová
doaj   +1 more source

Protein-bound molybdenum cofactor is bioavailable and rescues molybdenum cofactor-deficient C. elegans [PDF]

open access: yesGenes & Development, 2020
The molybdenum cofactor (Moco) is a 520-Da prosthetic group that is synthesized in all domains of life. In animals, four oxidases (among them sulfite oxidase) use Moco as a prosthetic group. Moco is essential in animals; humans with mutations in genes that encode Moco biosynthetic enzymes display lethal neurological and ...
Kurt Warnhoff   +3 more
openaire   +3 more sources

Dipstick Diagnosis of Neonatal Seizure Disorder

open access: yesPediatric Neurology Briefs, 1998
A dipstick test strip (Sulfistix) showing elevated urinary sulfite concentrations (400 mg/l) was used in diagnosis of molybdenum cofactor deficiency (MCD), an inborn errror of metabolism, in a male infant presenting with convulsions and cerebral ...
J Gordon Millichap
doaj   +1 more source

Copper-deficiency in Brassica napus induces copper remobilization, molybdenum accumulation and modification of the expression of chloroplastic proteins. [PDF]

open access: yesPLoS ONE, 2014
During the last 40 years, crop breeding has strongly increased yields but has had adverse effects on the content of micronutrients, such as Fe, Mg, Zn and Cu, in edible products despite their sufficient supply in most soils.
Vincent Billard   +9 more
doaj   +1 more source

The association of molybdenum cofactor deficiency and pyloric stenosis [PDF]

open access: yesJournal of Perinatology, 2012
Molybdenum cofactor deficiency (MoCD) is a rare autosomal recessive disorder that may present during the neonatal period with intractable seizures. Co-existence of MoCD and pyloric stenosis is previously reported as a coincidence or common etiology. The etiology of the two conditions is unclear; however, reports demonstrate neuronal deficiency in both.
G, Tezel   +5 more
openaire   +2 more sources

Importin 7 mediates the nuclear import of HIV‐1 integrase via a specific interacting interface

open access: yesFEBS Open Bio, EarlyView.
HIV‐1 integrase enables viral DNA integration into the host genome. By binding to the core domain of the host protein Importin 7 via its C‐terminal domain, the integrase is transported across the nuclear membrane into the nucleus, where integration of the viral genome into host DNA takes place. This translocation is a critical step for subsequent viral
Juana Bana   +5 more
wiley   +1 more source

The role of glutamate oxaloacetate transaminases in sulfite biosynthesis and H2S metabolism

open access: yesRedox Biology, 2021
Molybdenum cofactor deficiency and isolated sulfite oxidase deficiency are two rare genetic disorders that are caused by impairment of the mitochondrial enzyme sulfite oxidase.
Anna-Theresa Mellis   +8 more
doaj   +1 more source

Avidin is evolutionarily conserved in fish but dispensable for development and resistance against Streptococcus agalactiae in zebrafish

open access: yesFEBS Open Bio, EarlyView.
The presence of biotin‐binding avidin proteins in fish and their biological significance are poorly characterized. We cataloged fish avidins and demonstrate that they are widely present and evolutionarily conserved. We created avd knockout zebrafish and show that zebavidin is dispensable for development and that resistance of avd knockout embryos in ...
Anni K. Saralahti   +5 more
wiley   +1 more source

Mitochondria and Neuromast Tagging With Fluorescent Gallium‐Triapine Analogues: In Cellulo MP FLIM and Zebrafish Live Imaging

open access: yesAdvanced Science, EarlyView.
Fluorescent BODIPY‐conjugated thiosemicarbazone ligands and their Ga(III), In(III), and Fe(III) complexes, inspired by Triapine, are developed as theranostic agents. Multiphoton FLIM and confocal microscopy in cancer cells and zebrafish reveal real‐time uptake, mitochondrial localisation, and whilst spectroscopic assays indicated preserved complex ...
Megan J. Green   +15 more
wiley   +1 more source

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