PMID-sentid Pub_year Sent_text comp_official_name comp_offsetprotein_name organism prot_offset 31261385-1 2019 Pyridox (am) ine 5"-phosphate oxidase (PNPO) is a rate-limiting enzyme in converting dietary vitamin B6 (VB6) to pyridoxal 5"-phosphate (PLP), the biologically active form of VB6 and involved in the synthesis of neurotransmitters including gamma-aminobutyric acid (GABA), dopamine, and serotonin. Pyridoxal Phosphate 113-135 pyridoxamine 5'-phosphate oxidase Homo sapiens 39-43 29238081-1 2017 Pyridoxine 5"-phosphate oxidase (PNPO) is an enzyme that converts pyridoxine 5"-phosphate into pyridoxal 5"-phosphate (PLP), an active form of vitamin B6 implicated in several types of cancer. Pyridoxal Phosphate 95-117 pyridoxamine 5'-phosphate oxidase Homo sapiens 0-31 29238081-1 2017 Pyridoxine 5"-phosphate oxidase (PNPO) is an enzyme that converts pyridoxine 5"-phosphate into pyridoxal 5"-phosphate (PLP), an active form of vitamin B6 implicated in several types of cancer. Pyridoxal Phosphate 95-117 pyridoxamine 5'-phosphate oxidase Homo sapiens 33-37 28782931-5 2017 PNPO activity was quantified by measuring pyridoxal 5"-phosphate (PLP) concentrations in a DBS before and after a 30 min incubation with pyridoxine 5"-phosphate (PNP). Pyridoxal Phosphate 42-64 pyridoxamine 5'-phosphate oxidase Homo sapiens 0-4 28782931-5 2017 PNPO activity was quantified by measuring pyridoxal 5"-phosphate (PLP) concentrations in a DBS before and after a 30 min incubation with pyridoxine 5"-phosphate (PNP). Pyridoxal Phosphate 66-69 pyridoxamine 5'-phosphate oxidase Homo sapiens 0-4 28818555-1 2017 BACKGROUND: Pyridoxal-5"-phosphate oxidase (PNPO) deficiency presents as a severe neonatal encephalopathy responsive to pyridoxal-5"-phosphate (PLP) or pyridoxine. Pyridoxal Phosphate 120-142 pyridoxamine 5'-phosphate oxidase Homo sapiens 44-48 28779752-6 2017 Mutations in the gene encoding for the pyridoxal-5"-phosphate oxidase enzyme (PNPO) are responsible of a clinical entity similar to PDEs responsive to pyridoxal-5-phosphate administration not to pyridoxine administration. Pyridoxal Phosphate 151-172 pyridoxamine 5'-phosphate oxidase Homo sapiens 78-82 28133863-8 2017 The patient with the homozygous PNPO variant had a low cerebrospinal fluid pyridoxal-5-phosphate level. Pyridoxal Phosphate 75-96 pyridoxamine 5'-phosphate oxidase Homo sapiens 32-36 27419045-0 2016 Inactive mutants of human pyridoxine 5"-phosphate oxidase: a possible role for a noncatalytic pyridoxal 5"-phosphate tight binding site. Pyridoxal Phosphate 94-116 pyridoxamine 5'-phosphate oxidase Homo sapiens 26-57 26535729-0 2015 Pyridoxal 5 -phosphate-responsive epilepsy with novel mutations in the PNPO gene: a case report. Pyridoxal Phosphate 0-22 pyridoxamine 5'-phosphate oxidase Homo sapiens 71-75 24645144-3 2014 Sequencing of the PNPO gene was undertaken for a cohort of 82 individuals who had shown a reduction in frequency and severity of seizures in response to pyridoxine or pyridoxal 5"-phosphate. Pyridoxal Phosphate 167-189 pyridoxamine 5'-phosphate oxidase Homo sapiens 18-22 24645144-5 2014 Three groups of patients with PNPO mutations that had reduced enzyme activity were identified: (i) patients with neonatal onset seizures responding to pyridoxal 5"-phosphate (n = 6); (ii) a patient with infantile spasms (onset 5 months) responsive to pyridoxal 5"-phosphate (n = 1); and (iii) patients with seizures starting under 3 months of age responding to pyridoxine (n = 8). Pyridoxal Phosphate 151-173 pyridoxamine 5'-phosphate oxidase Homo sapiens 30-34 24645144-5 2014 Three groups of patients with PNPO mutations that had reduced enzyme activity were identified: (i) patients with neonatal onset seizures responding to pyridoxal 5"-phosphate (n = 6); (ii) a patient with infantile spasms (onset 5 months) responsive to pyridoxal 5"-phosphate (n = 1); and (iii) patients with seizures starting under 3 months of age responding to pyridoxine (n = 8). Pyridoxal Phosphate 251-273 pyridoxamine 5'-phosphate oxidase Homo sapiens 30-34 24645144-12 2014 Many of the mutations in PNPO affected residues involved in binding flavin mononucleotide or pyridoxal 5"-phosphate and many of them showed residual enzyme activity. Pyridoxal Phosphate 93-115 pyridoxamine 5'-phosphate oxidase Homo sapiens 25-29 24266778-1 2014 Pyridox(am)ine phosphate oxidase (PNPO) deficiency causes severe early infantile epileptic encephalopathy and has been characterized as responding to pyridoxal-5"-phosphate but not to pyridoxine. Pyridoxal Phosphate 150-172 pyridoxamine 5'-phosphate oxidase Homo sapiens 0-32 25256445-0 2014 Cirrhosis associated with pyridoxal 5"-phosphate treatment of pyridoxamine 5"-phosphate oxidase deficiency. Pyridoxal Phosphate 26-48 pyridoxamine 5'-phosphate oxidase Homo sapiens 62-95 23419474-2 2013 Pyridoxamine phosphate oxidase (PNPO) is one of a series of enzymes involved in converting pyridoxine to pyridoxal 5"-phosphate, the biologically active form of pyridoxine. Pyridoxal Phosphate 105-127 pyridoxamine 5'-phosphate oxidase Homo sapiens 0-30 23419474-2 2013 Pyridoxamine phosphate oxidase (PNPO) is one of a series of enzymes involved in converting pyridoxine to pyridoxal 5"-phosphate, the biologically active form of pyridoxine. Pyridoxal Phosphate 105-127 pyridoxamine 5'-phosphate oxidase Homo sapiens 32-36 20370816-3 2010 PDE and PNPO were proven by complete control of seizures once pyridoxine or pyridoxal 5"-phosphate was administered and by recurrence when withdrawn. Pyridoxal Phosphate 76-98 pyridoxamine 5'-phosphate oxidase Homo sapiens 8-12 20184790-10 2010 Pyridoxal phosphate-dependent seizures result from a deficiency of pyridox(am)ine 5"-phosphate oxidase which is encoded by PNPO. Pyridoxal Phosphate 0-19 pyridoxamine 5'-phosphate oxidase Homo sapiens 123-127 19759001-3 2009 Pyridoxine 5"-phosphate oxidase catalyzes the oxidation of pyridoxine 5"-phosphate to pyridoxal 5"-phosphate, the active cofactor form of vitamin B(6) required by more than 140 different catalytic activities, including enzymes involved in amino acid metabolism and biosynthesis of neurotransmitters. Pyridoxal Phosphate 86-108 pyridoxamine 5'-phosphate oxidase Homo sapiens 0-31 18024216-1 2008 We present a patient with severe pyridox(am)ine 5"-phosphate oxidase deficiency and homozygosity for a novel nonsense-mutation, p.A174X, in the PNPO gene who died with pyridoxal phosphate (PLP) treatment despite initial clinical recovery. Pyridoxal Phosphate 168-187 pyridoxamine 5'-phosphate oxidase Homo sapiens 144-148 16538088-6 2006 Other studies demonstrate that some children with intractable seizures respond to pyridoxal phosphate rather than pyridoxine, including a rare form of neonatal epileptic encephalopathy shown to be due to mutations in the PNPO gene for pyridox(am)ine 5"-phosphate oxidase. Pyridoxal Phosphate 82-101 pyridoxamine 5'-phosphate oxidase Homo sapiens 221-225 12824491-1 2003 Pyridoxine 5"-phosphate oxidase catalyzes the terminal step in the synthesis of pyridoxal 5"-phosphate. Pyridoxal Phosphate 80-102 pyridoxamine 5'-phosphate oxidase Homo sapiens 0-31 12111839-5 2002 The overexpression of PNP oxidase in the hippocampus of preseizure SS gerbils suggests that PNP or pyridoxal 5"-phosphate plays an important role in the modulation of glutamic acid decarboxylase activity and gamma-aminobutyric acid reuptake as mediated by membrane transporter via neurons. Pyridoxal Phosphate 99-121 pyridoxamine 5'-phosphate oxidase Homo sapiens 22-33 35050500-2 2022 Here, the non-coenzyme action of thiamine on the major mammalian producers of pyridoxal-5"-phosphate (PLP), such as pyridoxal kinase (PdxK) and pyridoxine 5"-phosphate oxidase (PNPO), is characterized. Pyridoxal Phosphate 78-100 pyridoxamine 5'-phosphate oxidase Homo sapiens 144-175 35050500-2 2022 Here, the non-coenzyme action of thiamine on the major mammalian producers of pyridoxal-5"-phosphate (PLP), such as pyridoxal kinase (PdxK) and pyridoxine 5"-phosphate oxidase (PNPO), is characterized. Pyridoxal Phosphate 78-100 pyridoxamine 5'-phosphate oxidase Homo sapiens 177-181 708447-0 1978 Evidence for the regulation of pyridoxal 5-phosphate formation in liver by pyridoxamine (pyridoxine) 5 -phosphate oxidase. Pyridoxal Phosphate 31-52 pyridoxamine 5'-phosphate oxidase Homo sapiens 75-121 32788630-4 2020 Here, we present a detailed characterisation of the inhibition mechanism of PNPO by pyridoxal 5"-phosphate (PLP), the reaction product of the enzyme. Pyridoxal Phosphate 84-106 pyridoxamine 5'-phosphate oxidase Homo sapiens 76-80