Results 41 to 50 of about 661,688 (260)
It takes two to tango: defining an essential second active site in pyridoxal 5'-phosphate synthase. [PDF]
PLoS ONE, 2011 The prevalent de novo biosynthetic pathway of vitamin B6 involves only two enzymes (Pdx1 and Pdx2) that form an ornate multisubunit complex functioning as a glutamine amidotransferase.
Cyril Moccand +2 more
doaj +1 more source
Effectiveness of Pyridoxal‐5′‐Phosphate in PNPO Deficiency: A Systematic Review [PDF]
J Inherit Metab DisABSTRACT Pyridox(am)ine 5′‐phosphate oxidase (PNPO) deficiency is an ultrarare inherited neurometabolic disease, characterized by primarily neonatal‐onset B6‐responsive epileptic encephalopathies. Treatment often requires sustainable access to high‐quality pyridoxal‐5′‐phosphate (PLP, i.e., active vitamin B6), although some patients (also) respond to ...
Stolwijk N +7 more
europepmc +2 more sources
Journal of General and Applied Microbiology, 2018 The gene ybhA of Escherichia coli encodes a phosphatase that has an in vitro specificity to dephosphorylate pyridoxal 5'-phosphate (PLP or vitamin B6), a co-factor for aminotransferases and other enzymes. In this study, we found that excess pyridoxal (PL)
Ryota Sugimoto +3 more
semanticscholar +1 more source
Pyridoxal Phosphate Supplementation in Neuropediatric Disorders
Seminars in Pediatric Neurology, 2016 Pyridoxal phosphate (PLP) is the active form of vitamin B6 and a cofactor in many enzyme reactions including neurotransmitter metabolism. PLP metabolism disturbances may mostly lead to refractory seizures. In this report, we review the main pathophysiological factors related with PLP deficiency and our experience in PLP treatment in pediatric patients ...
Elisenda Cortès-Saladelafont +4 more
openaire +3 more sources
Enzyme-independent catabolism of cysteine with pyridoxal-5′-phosphate
Scientific Reports, 2023 Pyridoxal-5′-phosphate (PLP) is a versatile cofactor that assists in different types of enzymatic reactions. PLP has also been reported to react with substrates and catalyze some of these reactions independent of enzymes.
Prajakatta Mulay +2 more
doaj +1 more source
Glutamate in Pyridoxine-Dependent Epilepsy
Pediatric Neurology Briefs, 1994 Cerebrospinal fluid levels of glutamate, g-aminobutyric acid, and pyridoxal-5-phosphate examined in a patient with pyridoxine dependency while on and off vitamin B6 treatment are reported from Universitat Munchen, and Universitats-Nervenklinik, Wurzburg,
J Gordon Millichap
doaj +1 more source
MOLECULAR MECHANISMS UNDERLYING THERAPEUTIC ACTION OF VITAMIN B6
Фармация и фармакология (Пятигорск), 2023 The aim of the study was to analyze the molecular mechanisms that determine the possibility of using vitamin B6 in clinical practice for the correction of various pathological conditions.Materials and methods.
O. A. Zagubnaya, Y. R. Nartsissov
doaj +1 more source
Hazelnut: Explorations toward the Biocatalytic Synthesis of its Aroma Precursor
ChemBioChem, EarlyView.This article proposes a biosynthetic route for synthesizing filbertone, the principal flavor compound of hazelnut, through a multienzyme cascade. The pathway uses D‐amino acid oxidase, threonine deaminase, and a regioselective transketolase from Geobacillus stearothermophilus to produce a sustainable, natural hazelnut aroma precursor, addressing ...
Mireia Salvadó‐Pau +3 more
wiley +1 more source
GABA Synthesis and Pyridoxine Seizures
Pediatric Neurology Briefs, 1994 A reduction in pyridoxal-5-phosphate (PLP) dependent enzyme, glutamic acid decarboxylase (GAD),which synthesizes GABA, is reported in a 3 month-old infant with seizures responsive to pyridoxine treated at University of California, Davis.
J Gordon Millichap
doaj +1 more source
Inherited metabolic epilepsies–established diseases, new approaches
Epilepsia Open, EarlyView.Abstract Inherited metabolic epilepsies (IMEs) represent the inherited metabolic disorders (IMDs) in which epilepsy is a prevailing component, often determining other neurodevelopmental outcomes associated with the disorder. The different metabolic pathways affected by individual IMEs are the basis of their rarity and heterogeneity.
Itay Tokatly Latzer, Phillip L. Pearl
wiley +1 more source