Activation of nicotinamide adenine dinucleotide phosphate oxidase is the primary trigger of epileptic seizures in rodent models
Sign Up to like & getrecommendations! Published in 2019 at "Annals of Neurology"
DOI: 10.1002/ana.25474
Abstract: Despite decades of epilepsy research, 30% of focal epilepsies remain resistant to antiseizure drugs, with effective drug development impeded by lack of understanding on how seizures are initiated. Here, we report the mechanism of seizure… read more here.
Keywords: adenine dinucleotide; nicotinamide adenine; dinucleotide phosphate; phosphate oxidase ... See more keywords
Phenotypic and molecular spectrum of pyridoxamine‐5′‐phosphate oxidase deficiency: A scoping review of 87 cases of pyridoxamine‐5′‐phosphate oxidase deficiency
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DOI: 10.1111/cge.13843
Abstract: Pyridoxamine‐5′‐phosphate oxidase (PNPO) deficiency is an autosomal recessive pyridoxal 5′‐phosphate (PLP)‐vitamin‐responsive epileptic encephalopathy. The emerging feature of PNPO deficiency is the occurrence of refractory seizures in the first year of life. Pre‐maturity and fetal distress,… read more here.
Keywords: pyridoxamine phosphate; oxidase deficiency; deficiency; phosphate oxidase ... See more keywords
Riboflavin kinase and pyridoxine 5′-phosphate oxidase complex formation envisages transient interactions for FMN cofactor delivery
Sign Up to like & getrecommendations! Published in 2023 at "Frontiers in Molecular Biosciences"
DOI: 10.3389/fmolb.2023.1167348
Abstract: Enzymes catalysing sequential reactions have developed different mechanisms to control the transport and flux of reactants and intermediates along metabolic pathways, which usually involve direct transfer of metabolites from an enzyme to the next one… read more here.
Keywords: interaction; phosphate oxidase; riboflavin kinase; pyridoxine phosphate ... See more keywords
Characterization of Novel Pathogenic Variants Causing Pyridox(am)ine 5′-Phosphate Oxidase-Dependent Epilepsy
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DOI: 10.3390/ijms222112013
Abstract: Several variants of the enzyme pyridox(am)ine 5′-phosphate oxidase (PNPO), responsible for a rare form of vitamin B6-dependent neonatal epileptic encephalopathy known as PNPO deficiency (PNPOD), have been reported. However, only a few of them have… read more here.
Keywords: pathogenic variants; pyridox ine; phosphate oxidase; ine phosphate ... See more keywords
Elucidating the Interaction between Pyridoxine 5′-Phosphate Oxidase and Dopa Decarboxylase: Activation of B6-Dependent Enzyme
Sign Up to like & getrecommendations! Published in 2022 at "International Journal of Molecular Sciences"
DOI: 10.3390/ijms24010642
Abstract: Pyridoxal 5′-phosphate (PLP), the active form of vitamin B6, serves as a cofactor for scores of B6-dependent (PLP-dependent) enzymes involved in many cellular processes. One such B6 enzyme is dopa decarboxylase (DDC), which is required… read more here.
Keywords: phosphate; phosphate oxidase; dopa decarboxylase; pyridoxine phosphate ... See more keywords