Page last updated: 2024-10-20

pyridoxal phosphate and Aura

pyridoxal phosphate has been researched along with Aura in 62 studies

Pyridoxal Phosphate: This is the active form of VITAMIN B 6 serving as a coenzyme for synthesis of amino acids, neurotransmitters (serotonin, norepinephrine), sphingolipids, aminolevulinic acid. During transamination of amino acids, pyridoxal phosphate is transiently converted into pyridoxamine phosphate (PYRIDOXAMINE).
pyridoxal 5'-phosphate : The monophosphate ester obtained by condensation of phosphoric acid with the primary hydroxy group of pyridoxal.

Research Excerpts

ExcerptRelevanceReference
"The study testifies an assumption on epilepsy as an inborn error of pyridoxine metabolism and suggests non-invasive quantitative biomarkers for clarified evaluation of clinical status and monitoring an individual treatment by antiepileptic drugs."9.16Epilepsy as a pyridoxine-dependent condition: quantified urinary biomarkers for status evaluation and monitoring antiepileptic treatment. ( Dolina, S; Malitsky, S; Margalit, D; Pressman, E; Rabinkov, A, 2012)
"To investigate the short-term efficacy and safety of high-dose pyridoxine and pyridoxal 5-phosphate (P5P) in the treatment of inherited glycosylphosphatidylinositol (GPI) deficiency-associated epilepsy."8.12Pyridoxine or pyridoxal-5-phosphate treatment for seizures in glycosylphosphatidylinositol deficiency: A cohort study. ( Aledo-Serrano, A; Bayat, A; Boßelmann, C; de Sain-van der Velden, MGM; Gardella, E; Gil-Nagel, A; Korff, CM; Lund, AM; Møller, RS; Thomas, A; Weber, Y, 2022)
"Using our laboratory database, we identified patients with vitamin B6-dependent epilepsy and extracted their data on the concentrations of pyridoxal 5'-phosphate, pyridoxal, pipecolic acid, α-aminoadipic semialdehyde, and monoamine neurotransmitters."7.96Pyridoxal in the Cerebrospinal Fluid May Be a Better Indicator of Vitamin B6-dependent Epilepsy Than Pyridoxal 5'-Phosphate. ( Akiyama, T; Baba, S; Dowa, Y; Fukuyama, T; Hamano, SI; Hasegawa, K; Hyodo, Y; Imai, K; Ishihara, N; Kobayashi, K; Koike, T; Kubota, M; Oboshi, T; Okanishi, T; Shibasaki, J; Shimbo, H; Shiraku, H; Takano, K; Takeshita, S; Yamamoto, T, 2020)
"α-Amino adipic semialdehyde (α-AASA) accumulates in body fluids from patients with pyridoxine-dependent epilepsy because of mutations in antiquitin (ALDH7A1) and serves as the biomarker for this condition."7.78Pyridoxine-dependent epilepsy with elevated urinary α-amino adipic semialdehyde in molybdenum cofactor deficiency. ( Al Shahwan, S; Bakkali, A; Nota, B; Salomons, GS; Struys, EA; Tabarki, B, 2012)
"Pyridoxine-dependent epilepsy (PDE) is a treatable inborn error of metabolism with autosomal recessive inheritance."7.77Status epilepticus in a neonate treated with pyridoxine because of a familial recurrence risk for antiquitin deficiency: pyridoxine toxicity? ( Fingerhut, M; Hartmann, H; Jakobs, C; Plecko, B, 2011)
"We report on seizures, paroxysmal events, and electroencephalogram (EEG) findings in four female infants with pyridoxine-dependent epilepsy (PDE) and in one female with pyridoxine phosphate oxidase deficiency (PNPO)."7.76Seizures and paroxysmal events: symptoms pointing to the diagnosis of pyridoxine-dependent epilepsy and pyridoxine phosphate oxidase deficiency. ( Baumgartner, M; Clayton, PT; Jakobs, C; Keller, E; Mills, PB; Schmitt, B; Wohlrab, G, 2010)
"Patients with pyridoxine dependent epilepsy (PDE) present with early-onset seizures resistant to common anticonvulsants."7.74Biochemical and molecular characterization of 18 patients with pyridoxine-dependent epilepsy and mutations of the antiquitin (ALDH7A1) gene. ( Baumeister, F; Bosch, F; di Capua, M; Erwa, W; Freilinger, M; Hartmann, H; Hikel, C; Jakobs, C; Korenke, C; Luecke, T; Paschke, E; Paul, K; Plecko, B; Reutershahn, E; Stoeckler-Ipsiroglu, S; Struys, E, 2007)
"To study the difference between pyridoxine (PN) and its active form, pyridoxal phosphate, (PLP) in control of idiopathic intractable epilepsy in children."7.73Pyridoxal phosphate is better than pyridoxine for controlling idiopathic intractable epilepsy. ( Chang, MY; Chou, ML; Hsieh, MY; Hung, PC; Kuo, MF; Lin, KL; Wang, HS, 2005)
"Diagnosis of pyridoxine-dependent epilepsy is based on the clinical response to high-dosage application of pyridoxine."7.70Pipecolic acid elevation in plasma and cerebrospinal fluid of two patients with pyridoxine-dependent epilepsy. ( Erwa, W; Jakobs, C; Paschke, E; Plecko, B; Stöckler-Ipsiroglu, S; Struys, EA, 2000)
"Pyridoxine-dependent epilepsy is a rare autosomal recessive disorder."7.69Glutamate in pyridoxine-dependent epilepsy: neurotoxic glutamate concentration in the cerebrospinal fluid and its normalization by pyridoxine. ( Baumeister, FA; Egger, J; Gsell, W; Shin, YS, 1994)
"Pyridoxal phosphate was extremely high in CSF and plasma."5.40Infantile hypophosphatasia without bone deformities presenting with severe pyridoxine-resistant seizures. ( Abeling, NGGM; Bosch, AM; Cobben, JM; de Roo, MGA; Duran, M; Koelman, JHTM; Majoie, CB; Poll-The, BT, 2014)
"The study testifies an assumption on epilepsy as an inborn error of pyridoxine metabolism and suggests non-invasive quantitative biomarkers for clarified evaluation of clinical status and monitoring an individual treatment by antiepileptic drugs."5.16Epilepsy as a pyridoxine-dependent condition: quantified urinary biomarkers for status evaluation and monitoring antiepileptic treatment. ( Dolina, S; Malitsky, S; Margalit, D; Pressman, E; Rabinkov, A, 2012)
" Antiquitin deficiency is the most common form of pyridoxine-dependent epilepsy."5.05Inherited Disorders of Lysine Metabolism: A Review. ( Bouchereau, J; Schiff, M, 2020)
" Pyridoxine dependent epilepsy is a treatable condition with a potentially widening clinical spectrum, but with a prognosis dependent on early intervention."4.82Clinical aspects of the disorders of GABA metabolism in children. ( Gibson, KM; Pearl, PL, 2004)
"To investigate the short-term efficacy and safety of high-dose pyridoxine and pyridoxal 5-phosphate (P5P) in the treatment of inherited glycosylphosphatidylinositol (GPI) deficiency-associated epilepsy."4.12Pyridoxine or pyridoxal-5-phosphate treatment for seizures in glycosylphosphatidylinositol deficiency: A cohort study. ( Aledo-Serrano, A; Bayat, A; Boßelmann, C; de Sain-van der Velden, MGM; Gardella, E; Gil-Nagel, A; Korff, CM; Lund, AM; Møller, RS; Thomas, A; Weber, Y, 2022)
"Using our laboratory database, we identified patients with vitamin B6-dependent epilepsy and extracted their data on the concentrations of pyridoxal 5'-phosphate, pyridoxal, pipecolic acid, α-aminoadipic semialdehyde, and monoamine neurotransmitters."3.96Pyridoxal in the Cerebrospinal Fluid May Be a Better Indicator of Vitamin B6-dependent Epilepsy Than Pyridoxal 5'-Phosphate. ( Akiyama, T; Baba, S; Dowa, Y; Fukuyama, T; Hamano, SI; Hasegawa, K; Hyodo, Y; Imai, K; Ishihara, N; Kobayashi, K; Koike, T; Kubota, M; Oboshi, T; Okanishi, T; Shibasaki, J; Shimbo, H; Shiraku, H; Takano, K; Takeshita, S; Yamamoto, T, 2020)
"We quantified pyridoxal 5'-phosphate (PLP), pyridoxal (PL), and 4-pyridoxic acid (PA) in the cerebrospinal fluid (CSF) of children and to investigate the effect of age, sex, epilepsy, and anti-epileptic drug (AED) therapy on these vitamers."3.85Measurement of pyridoxal 5'-phosphate, pyridoxal, and 4-pyridoxic acid in the cerebrospinal fluid of children. ( Akiyama, M; Akiyama, T; Hamano, SI; Hanaoka, Y; Hayashi, Y; Imai, K; Kobayashi, K; Okanishi, T; Shibata, T; Toda, S; Yoshinaga, H, 2017)
" Thiosemicarbazide (TSC) and γ-glutamyl-hydrazone (PLPGH) inhibit the free PLP-dependent isoform (GAD65) activity after systemic administration, leading to epilepsy in mice and in young, but not in adult rats."3.81Epilepsy and hippocampal neurodegeneration induced by glutamate decarboxylase inhibitors in awake rats. ( Salazar, P; Tapia, R, 2015)
"To determine whether patients with pyridoxine-responsive seizures but normal biomarkers for antiquitin deficiency and normal sequencing of the ALDH7A1 gene may have PNPO mutations."3.80Pyridoxine responsiveness in novel mutations of the PNPO gene. ( Abela, L; Clayton, P; Connolly, M; Hasselmann, O; Hofer, D; Kanz, S; Maier, O; Mills, P; Paschke, E; Paul, K; Plecko, B; Schmiedel, G; Stockler, S; Struys, E; Wolf, N, 2014)
"α-Amino adipic semialdehyde (α-AASA) accumulates in body fluids from patients with pyridoxine-dependent epilepsy because of mutations in antiquitin (ALDH7A1) and serves as the biomarker for this condition."3.78Pyridoxine-dependent epilepsy with elevated urinary α-amino adipic semialdehyde in molybdenum cofactor deficiency. ( Al Shahwan, S; Bakkali, A; Nota, B; Salomons, GS; Struys, EA; Tabarki, B, 2012)
"Pyridoxine-dependent epilepsy (PDE) is a treatable inborn error of metabolism with autosomal recessive inheritance."3.77Status epilepticus in a neonate treated with pyridoxine because of a familial recurrence risk for antiquitin deficiency: pyridoxine toxicity? ( Fingerhut, M; Hartmann, H; Jakobs, C; Plecko, B, 2011)
"We report on seizures, paroxysmal events, and electroencephalogram (EEG) findings in four female infants with pyridoxine-dependent epilepsy (PDE) and in one female with pyridoxine phosphate oxidase deficiency (PNPO)."3.76Seizures and paroxysmal events: symptoms pointing to the diagnosis of pyridoxine-dependent epilepsy and pyridoxine phosphate oxidase deficiency. ( Baumgartner, M; Clayton, PT; Jakobs, C; Keller, E; Mills, PB; Schmitt, B; Wohlrab, G, 2010)
"Patients with pyridoxine dependent epilepsy (PDE) present with early-onset seizures resistant to common anticonvulsants."3.74Biochemical and molecular characterization of 18 patients with pyridoxine-dependent epilepsy and mutations of the antiquitin (ALDH7A1) gene. ( Baumeister, F; Bosch, F; di Capua, M; Erwa, W; Freilinger, M; Hartmann, H; Hikel, C; Jakobs, C; Korenke, C; Luecke, T; Paschke, E; Paul, K; Plecko, B; Reutershahn, E; Stoeckler-Ipsiroglu, S; Struys, E, 2007)
"In the mouse, neurotransmitter metabolism can be regulated by modulation of the synthesis of pyridoxal 5'-phosphate and failure to maintain pyridoxal phosphate (PLP) levels results in epilepsy."3.73Neonatal epileptic encephalopathy caused by mutations in the PNPO gene encoding pyridox(am)ine 5'-phosphate oxidase. ( Beesley, CE; Briddon, A; Champion, MP; Clayton, PT; Dalton, N; Heales, SJ; Hoffmann, GF; Mills, PB; Scambler, PJ; Scheimberg, I; Surtees, RA; Zschocke, J, 2005)
"To study the difference between pyridoxine (PN) and its active form, pyridoxal phosphate, (PLP) in control of idiopathic intractable epilepsy in children."3.73Pyridoxal phosphate is better than pyridoxine for controlling idiopathic intractable epilepsy. ( Chang, MY; Chou, ML; Hsieh, MY; Hung, PC; Kuo, MF; Lin, KL; Wang, HS, 2005)
"Elevated concentrations of pipecolic acid have been reported in plasma and CSF of patients with pyridoxine-dependent epilepsy, but its molecular background is unclear."3.73Pipecolic acid concentrations in brain tissue of nutritionally pyridoxine-deficient rats. ( Hoeger, H; Jakobs, C; Leschnik, M; Muehl, A; Plecko, B; Stoeckler-Ipsiroglu, S; Stromberger, C; Struys, E, 2005)
"Diagnosis of pyridoxine-dependent epilepsy is based on the clinical response to high-dosage application of pyridoxine."3.70Pipecolic acid elevation in plasma and cerebrospinal fluid of two patients with pyridoxine-dependent epilepsy. ( Erwa, W; Jakobs, C; Paschke, E; Plecko, B; Stöckler-Ipsiroglu, S; Struys, EA, 2000)
"Pyridoxine-dependent epilepsy is a rare autosomal recessive disorder."3.69Glutamate in pyridoxine-dependent epilepsy: neurotoxic glutamate concentration in the cerebrospinal fluid and its normalization by pyridoxine. ( Baumeister, FA; Egger, J; Gsell, W; Shin, YS, 1994)
"In spite of effective seizure control with PLP, approximately 56% of patients affected with PLP-dependent epilepsy suffer developmental delay/intellectual disability."2.72Phenotypic and molecular spectrum of pyridoxamine-5'-phosphate oxidase deficiency: A scoping review of 87 cases of pyridoxamine-5'-phosphate oxidase deficiency. ( Abdelhakim, M; Adly, N; Alghamdi, M; Alghanem, B; Arold, ST; Bashiri, FA; Jamjoom, DZ; Sumaily, KM, 2021)
"Treatment with pyridoxine significantly improved the epileptic phenotype and extended lifespan in plpbp-/- animals."1.51PLPHP deficiency: clinical, genetic, biochemical, and mechanistic insights. ( Abdelrahim, RA; Al Futaisi, A; Al-Shekaili, HH; Al-Thihli, K; Arnold, GL; Ban, K; Begtrup, A; Boon, M; Bosma, M; Boycott, KM; Brimble, E; Bui, T; Cho, MT; Ciapaite, J; Demarest, S; Drögemöller, B; Dyment, DA; Ekker, M; Friedman, JM; Gerkes, EH; Haaxma, CA; Heiner-Fokkema, MR; Houten, SM; Ito, Y; Ivy, AS; Jans, J; Johnstone, DL; Kamsteeg, EJ; Kema, IP; Kernohan, KD; Koolen, DA; Kosuta, C; Koul, R; Lepage, N; Lines, MA; Majewski, J; McBride, S; Noble, S; Olson, H; Pena, IA; Ross, CJ; Roussel, Y; Sigurdardottir, LY; Tarailo-Graovac, M; van Faassen, M; van Karnebeek, CDM; van Roermund, CWT; Verhoeven-Duif, N; Violante, S; Wanders, RJA; Wasserman, WW; Wevers, RA; Wolf, NI, 2019)
"Pyridoxal phosphate was extremely high in CSF and plasma."1.40Infantile hypophosphatasia without bone deformities presenting with severe pyridoxine-resistant seizures. ( Abeling, NGGM; Bosch, AM; Cobben, JM; de Roo, MGA; Duran, M; Koelman, JHTM; Majoie, CB; Poll-The, BT, 2014)
"Vitamin B(6) dependent seizure disorders are an important and treatable cause of childhood epilepsy."1.39Measurement of plasma B6 vitamer profiles in children with inborn errors of vitamin B6 metabolism using an LC-MS/MS method. ( Clayton, PT; Footitt, EJ; Heales, SJ; Mills, K; Mills, PB; Neergheen, V; Oppenheim, M, 2013)
"Neonatal epileptic encephalopathy can be caused by inborn errors of metabolism."1.34Pyridoxal 5'-phosphate may be curative in early-onset epileptic encephalopathy. ( Bagci, S; Bast, T; Baumgartner, MR; Clayton, PT; Franz, AR; Hoffmann, GF; Mills, PB; Schmitt, B; Steinmann, B; Strehl, H; Wagner, N; Windfuhr, M; Wolf, NI; Zschocke, J, 2007)

Research

Studies (62)

TimeframeStudies, this research(%)All Research%
pre-199013 (20.97)18.7374
1990's3 (4.84)18.2507
2000's17 (27.42)29.6817
2010's22 (35.48)24.3611
2020's7 (11.29)2.80

Authors

AuthorsStudies
Barile, A1
Mills, P2
di Salvo, ML1
Graziani, C1
Bunik, V1
Clayton, P2
Contestabile, R1
Tramonti, A1
Bayat, A1
Aledo-Serrano, A1
Gil-Nagel, A1
Korff, CM1
Thomas, A1
Boßelmann, C1
Weber, Y1
Gardella, E1
Lund, AM1
de Sain-van der Velden, MGM1
Møller, RS1
Plecko, B7
Alghamdi, M1
Bashiri, FA1
Abdelhakim, M1
Adly, N1
Jamjoom, DZ1
Sumaily, KM1
Alghanem, B1
Arold, ST1
Akiyama, T2
Hyodo, Y1
Hasegawa, K1
Oboshi, T1
Imai, K2
Ishihara, N1
Dowa, Y1
Koike, T1
Yamamoto, T1
Shibasaki, J1
Shimbo, H1
Fukuyama, T1
Takano, K1
Shiraku, H1
Takeshita, S1
Okanishi, T2
Baba, S1
Kubota, M1
Hamano, SI2
Kobayashi, K2
Bouchereau, J1
Schiff, M2
Klotz, KA1
Lemke, JR1
Korinthenberg, R1
Jacobs, J1
Wilson, MP2
Footitt, EJ5
Papandreou, A1
Uudelepp, ML1
Pressler, R1
Stevenson, DC1
Gabriel, C1
McSweeney, M1
Baggot, M1
Burke, D1
Stödberg, T2
Riney, K2
Heales, SJR1
Mills, KA2
Gissen, P2
Clayton, PT9
Mills, PB8
Tremiño, L1
Forcada-Nadal, A1
Contreras, A1
Rubio, V1
Guerriero, RM1
Patel, AA1
Walsh, B1
Baumer, FM1
Shah, AS1
Peters, JM1
Rodan, LH1
Agrawal, PB1
Pearl, PL3
Takeoka, M1
Johnstone, DL1
Al-Shekaili, HH1
Tarailo-Graovac, M1
Wolf, NI3
Ivy, AS1
Demarest, S1
Roussel, Y1
Ciapaite, J1
van Roermund, CWT1
Kernohan, KD1
Kosuta, C1
Ban, K1
Ito, Y1
McBride, S1
Al-Thihli, K1
Abdelrahim, RA1
Koul, R1
Al Futaisi, A1
Haaxma, CA1
Olson, H1
Sigurdardottir, LY1
Arnold, GL1
Gerkes, EH1
Boon, M1
Heiner-Fokkema, MR1
Noble, S1
Bosma, M1
Jans, J1
Koolen, DA1
Kamsteeg, EJ1
Drögemöller, B1
Ross, CJ1
Majewski, J1
Cho, MT1
Begtrup, A1
Wasserman, WW1
Bui, T1
Brimble, E1
Violante, S1
Houten, SM1
Wevers, RA1
van Faassen, M1
Kema, IP1
Lepage, N1
Lines, MA1
Dyment, DA1
Wanders, RJA1
Verhoeven-Duif, N1
Ekker, M1
Boycott, KM1
Friedman, JM1
Pena, IA2
van Karnebeek, CDM1
de Roo, MGA1
Abeling, NGGM1
Majoie, CB1
Bosch, AM1
Koelman, JHTM1
Cobben, JM1
Duran, M1
Poll-The, BT1
Camuzeaux, SS1
Fisher, L1
Das, KB1
Varadkar, SM1
Zuberi, S1
McWilliam, R1
Baumgartner, MR2
Maier, O2
Calvert, S1
Livingston, JH1
Bala, P1
Morel, CF1
Feillet, F1
Raimondi, F1
Del Giudice, E1
Chong, WK1
Pitt, M1
Paul, K2
Paschke, E3
Hasselmann, O1
Schmiedel, G1
Kanz, S1
Connolly, M1
Wolf, N2
Struys, E3
Stockler, S1
Abela, L1
Hofer, D1
Dietmann, A1
Wallner, B1
König, R1
Friedrich, K1
Pfausler, B1
Deisenhammer, F1
Griesmacher, A1
Seger, C1
Matuja, W1
JilekAall, L1
Winkler, AS1
Schmutzhard, E1
Salazar, P1
Tapia, R1
Marques, LA1
Laranjeira, ÂB1
Yunes, JA1
Eberlin, MN1
MacKenzie, A1
Arruda, P1
Akiyama, M1
Hayashi, Y1
Shibata, T1
Hanaoka, Y1
Toda, S1
Yoshinaga, H1
Mohamed-Ahmed, AH1
Albuera, M1
Chen, T1
Tuleu, C1
Cortès-Saladelafont, E1
Molero-Luis, M1
Artuch, R2
García-Cazorla, À1
Gospe, SM1
Schmitt, B2
Baumgartner, M1
Jakobs, C6
Keller, E1
Wohlrab, G1
Veerapandiyan, A1
Winchester, SA1
Gallentine, WB1
Smith, EC1
Kansagra, S1
Hyland, K1
Mikati, MA1
Heales, SJ3
Allen, GF1
Oppenheim, M2
Hartmann, H2
Fingerhut, M1
Dill, P1
Schneider, J1
Weber, P1
Trachsel, D1
Tekin, M1
Thöny, B1
Blau, N1
Mills, K1
Neergheen, V1
Dolina, S1
Margalit, D1
Malitsky, S1
Pressman, E1
Rabinkov, A1
Struys, EA2
Nota, B1
Bakkali, A1
Al Shahwan, S1
Salomons, GS1
Tabarki, B1
Wang, H1
Kuo, M1
Gibson, KM1
Tutor-Crespo, MJ2
Hermida, J2
Tutor, JC2
Surtees, RA1
Champion, MP1
Beesley, CE1
Dalton, N1
Scambler, PJ1
Briddon, A1
Scheimberg, I1
Hoffmann, GF3
Zschocke, J4
Wang, HS1
Kuo, MF1
Chou, ML1
Hung, PC1
Lin, KL1
Hsieh, MY1
Chang, MY1
Hoeger, H1
Stromberger, C1
Leschnik, M1
Muehl, A1
Stoeckler-Ipsiroglu, S2
Sener, U1
Zorlu, Y1
Karaguzel, O1
Ozdamar, O1
Coker, I1
Topbas, M1
Attilakos, A1
Papakonstantinou, E1
Schulpis, K1
Voudris, K1
Katsarou, E1
Mastroyianni, S1
Garoufi, A1
Luecke, T1
di Capua, M1
Korenke, C1
Hikel, C1
Reutershahn, E1
Freilinger, M1
Baumeister, F1
Bosch, F1
Erwa, W2
Windfuhr, M1
Wagner, N1
Strehl, H1
Bagci, S2
Franz, AR2
Steinmann, B1
Bast, T2
Surtees, R1
Ruiz, A1
García-Villoria, J1
Ormazabal, A1
Fiol, M1
Navarro-Sastre, A1
Vilaseca, MA1
Ribes, A1
Klepper, J1
Müller, A1
Heep, A1
Bartmann, P1
Kang, TC1
Kang, JH1
Kim, HT1
Lee, SJ1
Choi, UK1
Kim, JE1
Kwak, SE1
Kim, DW1
Choi, SY1
Kwon, OS1
Chung, SH2
Johnson, MS1
Shandra, AA2
Kryzhanovskiĭ, GN2
Godlevskiĭ, LS1
Hammad, HM1
Al-Sayegh, A1
Swanson, S1
Ebadi, M2
Kouyoumdjian, JC1
Waymire, KG1
Mahuren, JD1
Jaje, JM1
Guilarte, TR1
Coburn, SP1
MacGregor, GR1
Baumeister, FA1
Gsell, W1
Shin, YS1
Egger, J1
Ross, FM1
Brodie, MJ1
Stone, TW1
Stöckler-Ipsiroglu, S1
Lott, IT1
Coulombe, T1
Di Paolo, RV1
Richardson, EP1
Levy, HL1
Reinken, L1
Gabrielsson, B1
Norris, DK1
Krause, KH1
Bonjour, JP1
Berlit, P1
Kynast, G1
Schmidt-Gayk, H1
Arab, L1
Papeschi, R1
Molina-Negro, P1
Sourkes, TL1
Erba, G1
Antón-Tay, F1
Hagberg, B2
Hamfelt, A1
Hansson, O2

Reviews

8 reviews available for pyridoxal phosphate and Aura

ArticleYear
On pathways and blind alleys-The importance of biomarkers in vitamin B
    Journal of inherited metabolic disease, 2023, Volume: 46, Issue:5

    Topics: Biomarkers; Epilepsy; Humans; Infant, Newborn; Pyridoxal Phosphate; Pyridoxine; Vitamin B 6; Vitamin

2023
Phenotypic and molecular spectrum of pyridoxamine-5'-phosphate oxidase deficiency: A scoping review of 87 cases of pyridoxamine-5'-phosphate oxidase deficiency.
    Clinical genetics, 2021, Volume: 99, Issue:1

    Topics: Brain Diseases, Metabolic; Epilepsy; Humans; Hypoxia-Ischemia, Brain; Metabolic Diseases; Mutation;

2021
Inherited Disorders of Lysine Metabolism: A Review.
    The Journal of nutrition, 2020, 10-01, Volume: 150, Issue:Suppl 1

    Topics: 2-Aminoadipic Acid; Aldehyde Dehydrogenase; Amino Acid Metabolism, Inborn Errors; Arginine; Brain; B

2020
Electroencephalographic and seizure manifestations of pyridoxal 5'-phosphate-dependent epilepsy.
    Epilepsy & behavior : E&B, 2011, Volume: 20, Issue:3

    Topics: Child, Preschool; Electroencephalography; Epilepsy; Humans; Infant; Male; Mutation; Pyridoxal Phosph

2011
Clinical aspects of the disorders of GABA metabolism in children.
    Current opinion in neurology, 2004, Volume: 17, Issue:2

    Topics: 4-Aminobutyrate Transaminase; Aldehyde Oxidoreductases; Amino Acid Metabolism, Inborn Errors; Brain;

2004
Studies on sound-induced epilepsy in mice.
    Proceedings of the Royal Society of London. Series B, Biological sciences, 1984, Apr-24, Volume: 221, Issue:1223

    Topics: Acoustic Stimulation; Amino Acids; Animals; Brain; Copper; Epilepsy; Female; Male; Mice; Mice, Inbre

1984
Transition metal ions in epilepsy: an overview.
    Advances in experimental medicine and biology, 1986, Volume: 203

    Topics: Amino Acids; Animals; Biological Transport; Cations, Divalent; Enzymes; Epilepsy; Extracellular Spac

1986
Melatonin: effects on brain function.
    Advances in biochemical psychopharmacology, 1974, Volume: 11, Issue:0

    Topics: Adenylyl Cyclases; Aminobutyrates; Animals; Behavior; Brain; Cerebral Cortex; Cyclic AMP; Electric S

1974

Trials

1 trial available for pyridoxal phosphate and Aura

ArticleYear
Epilepsy as a pyridoxine-dependent condition: quantified urinary biomarkers for status evaluation and monitoring antiepileptic treatment.
    Medical hypotheses, 2012, Volume: 79, Issue:2

    Topics: Adolescent; Anticonvulsants; Biomarkers; Child; Child, Preschool; Epilepsy; Female; Humans; Male; Py

2012

Other Studies

53 other studies available for pyridoxal phosphate and Aura

ArticleYear
Characterization of Novel Pathogenic Variants Causing Pyridox(am)ine 5'-Phosphate Oxidase-Dependent Epilepsy.
    International journal of molecular sciences, 2021, Nov-06, Volume: 22, Issue:21

    Topics: Brain Diseases, Metabolic; Epilepsy; Humans; Hypoxia-Ischemia, Brain; Infant, Newborn; Metabolic Dis

2021
Pyridoxine or pyridoxal-5-phosphate treatment for seizures in glycosylphosphatidylinositol deficiency: A cohort study.
    Developmental medicine and child neurology, 2022, Volume: 64, Issue:6

    Topics: Cohort Studies; Drug Resistant Epilepsy; Epilepsy; Female; Glycosylphosphatidylinositols; Humans; In

2022
Pyridoxal in the Cerebrospinal Fluid May Be a Better Indicator of Vitamin B6-dependent Epilepsy Than Pyridoxal 5'-Phosphate.
    Pediatric neurology, 2020, Volume: 113

    Topics: 5-Hydroxytryptophan; Adolescent; Child; Child, Preschool; Epilepsy; Female; Humans; Infant; Infant,

2020
Vitamin B6-Responsive Epilepsy due to a Novel KCNQ2 Mutation.
    Neuropediatrics, 2017, Volume: 48, Issue:3

    Topics: Anticonvulsants; Brain; Epilepsy; Female; Humans; Infant; KCNQ2 Potassium Channel; Mutation; Pyridox

2017
An LC-MS/MS-Based Method for the Quantification of Pyridox(am)ine 5'-Phosphate Oxidase Activity in Dried Blood Spots from Patients with Epilepsy.
    Analytical chemistry, 2017, 09-05, Volume: 89, Issue:17

    Topics: Adolescent; Adult; Area Under Curve; Case-Control Studies; Child; Child, Preschool; Chromatography,

2017
Studies on cyanobacterial protein PipY shed light on structure, potential functions, and vitamin B
    FEBS letters, 2017, Volume: 591, Issue:20

    Topics: Amino Acid Motifs; Bacterial Proteins; Binding Sites; Cloning, Molecular; Crystallography, X-Ray; Cy

2017
Systemic Manifestations in Pyridox(am)ine 5'-Phosphate Oxidase Deficiency.
    Pediatric neurology, 2017, Volume: 76

    Topics: Brain Diseases, Metabolic; Electroencephalography; Epilepsy; Female; Humans; Hypoxia-Ischemia, Brain

2017
PLPHP deficiency: clinical, genetic, biochemical, and mechanistic insights.
    Brain : a journal of neurology, 2019, 03-01, Volume: 142, Issue:3

    Topics: Animals; Disease Models, Animal; Epilepsy; Female; HEK293 Cells; Humans; Male; Phenotype; Proteins;

2019
The effectiveness of correcting abnormal metabolic profiles.
    Journal of inherited metabolic disease, 2020, Volume: 43, Issue:1

    Topics: 3-Hydroxysteroid Dehydrogenases; Administration, Oral; Bile Acids and Salts; Epilepsy; Humans; Metab

2020
Infantile hypophosphatasia without bone deformities presenting with severe pyridoxine-resistant seizures.
    Molecular genetics and metabolism, 2014, Volume: 111, Issue:3

    Topics: Alkaline Phosphatase; Drug Resistance; Epilepsy; Humans; Hypophosphatasia; Infant; Male; Pyridoxal P

2014
Epilepsy due to PNPO mutations: genotype, environment and treatment affect presentation and outcome.
    Brain : a journal of neurology, 2014, Volume: 137, Issue:Pt 5

    Topics: Anticonvulsants; Child; Child, Preschool; Electroencephalography; Environment; Epilepsy; Female; HeL

2014
Pyridoxine responsiveness in novel mutations of the PNPO gene.
    Neurology, 2014, Apr-22, Volume: 82, Issue:16

    Topics: Aldehyde Dehydrogenase; Alleles; Animals; Brain Diseases, Metabolic; CHO Cells; Chromosome Deletion;

2014
Nodding syndrome in Tanzania may not be associated with circulating anti-NMDA-and anti-VGKC receptor antibodies or decreased pyridoxal phosphate serum levels-a pilot study.
    African health sciences, 2014, Volume: 14, Issue:2

    Topics: Adolescent; Adult; Aged; Autoantibodies; Case-Control Studies; Epilepsy; Female; Humans; Male; Middl

2014
Epilepsy and hippocampal neurodegeneration induced by glutamate decarboxylase inhibitors in awake rats.
    Epilepsy research, 2015, Volume: 116

    Topics: Amino Acids; Animals; Disease Models, Animal; Dizocilpine Maleate; Dose-Response Relationship, Drug;

2015
Mouse lysine catabolism to aminoadipate occurs primarily through the saccharopine pathway; implications for pyridoxine dependent epilepsy (PDE).
    Biochimica et biophysica acta. Molecular basis of disease, 2017, Volume: 1863, Issue:1

    Topics: 2-Aminoadipic Acid; Animals; Epilepsy; Female; Lysine; Metabolic Networks and Pathways; Mice; Mice,

2017
Measurement of pyridoxal 5'-phosphate, pyridoxal, and 4-pyridoxic acid in the cerebrospinal fluid of children.
    Clinica chimica acta; international journal of clinical chemistry, 2017, Volume: 466

    Topics: Age Factors; Child; Chromatography, High Pressure Liquid; Epilepsy; Female; Humans; Linear Models; M

2017
Quality and stability of extemporaneous pyridoxal phosphate preparations used in the treatment of paediatric epilepsy.
    The Journal of pharmacy and pharmacology, 2017, Volume: 69, Issue:4

    Topics: Dietary Supplements; Dosage Forms; Drug Stability; Drug Storage; Epilepsy; Pharmaceutical Solutions;

2017
Pyridoxal Phosphate Supplementation in Neuropediatric Disorders.
    Seminars in pediatric neurology, 2016, Volume: 23, Issue:4

    Topics: Adolescent; Child; Child, Preschool; Epilepsy; Humans; Infant; Pyridoxal Phosphate; Treatment Outcom

2016
Pyridoxal phosphate dependency, a newly recognized treatable catastrophic epileptic encephalopathy.
    Journal of inherited metabolic disease, 2007, Volume: 30, Issue:1

    Topics: Aldehyde Dehydrogenase; Epilepsy; Humans; Infant, Newborn; Metabolism, Inborn Errors; Pyridoxal Phos

2007
Seizures and paroxysmal events: symptoms pointing to the diagnosis of pyridoxine-dependent epilepsy and pyridoxine phosphate oxidase deficiency.
    Developmental medicine and child neurology, 2010, Volume: 52, Issue:7

    Topics: Aldehyde Dehydrogenase; Anticonvulsants; Brain; Brain Diseases, Metabolic; Diagnosis, Differential;

2010
Pyridoxal 5'-phosphate in cerebrospinal fluid; factors affecting concentration.
    Journal of inherited metabolic disease, 2011, Volume: 34, Issue:2

    Topics: Adolescent; Adult; Biopterins; Child; Child, Preschool; Cysteine; Epilepsy; False Positive Reactions

2011
Status epilepticus in a neonate treated with pyridoxine because of a familial recurrence risk for antiquitin deficiency: pyridoxine toxicity?
    Developmental medicine and child neurology, 2011, Volume: 53, Issue:12

    Topics: Adult; Aldehyde Dehydrogenase; Epilepsy; Female; Humans; Infant; Infant, Newborn; Infusions, Parente

2011
Pyridoxal phosphate-responsive seizures in a patient with cerebral folate deficiency (CFD) and congenital deafness with labyrinthine aplasia, microtia and microdontia (LAMM).
    Molecular genetics and metabolism, 2011, Volume: 104, Issue:3

    Topics: Base Sequence; Child; Codon, Nonsense; Congenital Abnormalities; Congenital Microtia; Dihydroxypheny

2011
Measurement of plasma B6 vitamer profiles in children with inborn errors of vitamin B6 metabolism using an LC-MS/MS method.
    Journal of inherited metabolic disease, 2013, Volume: 36, Issue:1

    Topics: Adolescent; Child; Child, Preschool; Chromatography, Liquid; Epilepsy; Humans; Metabolism, Inborn Er

2013
Pyridoxine-dependent epilepsy with elevated urinary α-amino adipic semialdehyde in molybdenum cofactor deficiency.
    Pediatrics, 2012, Volume: 130, Issue:6

    Topics: 2-Aminoadipic Acid; Aldehyde Dehydrogenase; Brain; Child, Preschool; Consanguinity; Developmental Di

2012
Pyridoxine sometimes fails to be activated to pyridoxal phosphate.
    Brain research bulletin, 2002, Sep-30, Volume: 58, Issue:6

    Topics: Epilepsy; Humans; Pyridoxal Phosphate; Pyridoxine

2002
Activation of serum aminotransferases by pyridoxal-5' -phosphate in epileptic patients treated with anticonvulsant drugs.
    Clinical biochemistry, 2004, Volume: 37, Issue:8

    Topics: Adult; Alanine Transaminase; Aspartate Aminotransferases; Carbamazepine; Drug Therapy, Combination;

2004
Neonatal epileptic encephalopathy caused by mutations in the PNPO gene encoding pyridox(am)ine 5'-phosphate oxidase.
    Human molecular genetics, 2005, Apr-15, Volume: 14, Issue:8

    Topics: Amino Acid Sequence; Animals; Base Sequence; CHO Cells; Cricetinae; Cricetulus; DNA Mutational Analy

2005
Pyridoxal phosphate is better than pyridoxine for controlling idiopathic intractable epilepsy.
    Archives of disease in childhood, 2005, Volume: 90, Issue:5

    Topics: Administration, Oral; Adolescent; Anticonvulsants; Child; Child, Preschool; Drug Administration Sche

2005
Pipecolic acid concentrations in brain tissue of nutritionally pyridoxine-deficient rats.
    Journal of inherited metabolic disease, 2005, Volume: 28, Issue:5

    Topics: Animal Nutritional Physiological Phenomena; Animals; Brain; Disease Models, Animal; Epilepsy; Female

2005
Effect of antiepileptic drugs on the urinary excretion of porphyrins in non-porphyric subjects.
    Journal of pharmacological sciences, 2005, Volume: 99, Issue:4

    Topics: Adult; Anticonvulsants; Cholestasis; Enzyme Induction; Enzymes; Epilepsy; Female; Glucaric Acid; Hum

2005
Effects of common anti-epileptic drug monotherapy on serum levels of homocysteine, vitamin B12, folic acid and vitamin B6.
    Seizure, 2006, Volume: 15, Issue:2

    Topics: Adolescent; Adult; Anticonvulsants; Carbamazepine; Case-Control Studies; Epilepsy; Female; Folic Aci

2006
Early effect of sodium valproate and carbamazepine monotherapy on homocysteine metabolism in children with epilepsy.
    Epilepsy research, 2006, Volume: 71, Issue:2-3

    Topics: Adolescent; Anticonvulsants; Carbamazepine; Child; Child, Preschool; Epilepsy; Female; Folic Acid; H

2006
Biochemical and molecular characterization of 18 patients with pyridoxine-dependent epilepsy and mutations of the antiquitin (ALDH7A1) gene.
    Human mutation, 2007, Volume: 28, Issue:1

    Topics: Aldehyde Dehydrogenase; Amino Acid Sequence; DNA Mutational Analysis; Epilepsy; Female; Humans; Infa

2007
Pyridoxal 5'-phosphate may be curative in early-onset epileptic encephalopathy.
    Journal of inherited metabolic disease, 2007, Volume: 30, Issue:1

    Topics: Age of Onset; Brain Diseases; Child, Preschool; Epilepsy; Female; Humans; Infant; Infant, Newborn; M

2007
Treatable neonatal epilepsy.
    Archives of disease in childhood, 2007, Volume: 92, Issue:8

    Topics: Biomarkers; Epilepsy; Humans; Infant, Newborn; Infant, Newborn, Diseases; Leucovorin; Metabolic Dise

2007
A new fatal case of pyridox(am)ine 5'-phosphate oxidase (PNPO) deficiency.
    Molecular genetics and metabolism, 2008, Volume: 93, Issue:2

    Topics: Brain Diseases, Metabolic, Inborn; Codon, Nonsense; Epilepsy; Fatal Outcome; Genes, Recessive; Homov

2008
Pyridoxal phosphate-dependent neonatal epileptic encephalopathy.
    Archives of disease in childhood. Fetal and neonatal edition, 2008, Volume: 93, Issue:2

    Topics: Brain Diseases; Child, Preschool; Consanguinity; DNA Mutational Analysis; Electroencephalography; Ep

2008
Anticonvulsant characteristics of pyridoxyl-gamma-aminobutyrate, PL-GABA.
    Neuropharmacology, 2008, Volume: 54, Issue:6

    Topics: 3-Mercaptopropionic Acid; Animals; Anticonvulsants; Bicuculline; Drug Tolerance; Electrophysiology;

2008
[Antiepileptic effects of synthetic pharmacologic agents and vitamin preparations used in combination].
    Zhurnal nevropatologii i psikhiatrii imeni S.S. Korsakova (Moscow, Russia : 1952), 1984, Volume: 84, Issue:6

    Topics: Animals; Anticonvulsants; Crosses, Genetic; Diazepam; Drug Therapy, Combination; Epilepsy; Mice; Mic

1984
[Effect of vitamin preparations on epileptic activity].
    Biulleten' eksperimental'noi biologii i meditsiny, 1984, Volume: 98, Issue:8

    Topics: Animals; Drug Evaluation, Preclinical; Drug Therapy, Combination; Epilepsy; Kindling, Neurologic; Mi

1984
Dissociation between epileptic seizures induced by convulsant drugs and alteration in the concentrations of pyridoxal phosphate in rat brain regions.
    General pharmacology, 1983, Volume: 14, Issue:5

    Topics: 3-Mercaptopropionic Acid; Allylglycine; Animals; Bicuculline; Brain; Convulsants; Epilepsy; Male; Pi

1983
Anticonvulsant activity of muscimol and gamma-aminobutyric acid against pyridoxal phosphate-induced epileptic seizures.
    Journal of neurochemistry, 1981, Volume: 36, Issue:1

    Topics: Animals; Brain; Dose-Response Relationship, Drug; Epilepsy; gamma-Aminobutyric Acid; Glutamate Decar

1981
Mice lacking tissue non-specific alkaline phosphatase die from seizures due to defective metabolism of vitamin B-6.
    Nature genetics, 1995, Volume: 11, Issue:1

    Topics: Alkaline Phosphatase; Animals; Brain Chemistry; Calcification, Physiologic; Disease Models, Animal;

1995
Glutamate in pyridoxine-dependent epilepsy: neurotoxic glutamate concentration in the cerebrospinal fluid and its normalization by pyridoxine.
    Pediatrics, 1994, Volume: 94, Issue:3

    Topics: Adrenocorticotropic Hormone; Electroencephalography; Epilepsy; gamma-Aminobutyric Acid; Genes, Reces

1994
Modulation by adenine nucleotides of epileptiform activity in the CA3 region of rat hippocampal slices.
    British journal of pharmacology, 1998, Volume: 123, Issue:1

    Topics: Adenine Nucleotides; Adenosine; Adenosine Deaminase; Adenosine Triphosphate; Animals; Electric Stimu

1998
Pipecolic acid elevation in plasma and cerebrospinal fluid of two patients with pyridoxine-dependent epilepsy.
    Annals of neurology, 2000, Volume: 48, Issue:1

    Topics: 2-Aminoadipic Acid; Child; Epilepsy; Humans; Infant, Newborn; Male; Picolinic Acids; Pipecolic Acids

2000
Vitamin B6-dependent seizures: pathology and chemical findings in brain.
    Neurology, 1978, Volume: 28, Issue:1

    Topics: Adolescent; Adult; Amino Acids; Brain; Cerebellar Cortex; Cerebral Cortex; Child; Child, Preschool;

1978
The influence of antiepileptic drugs on vitamin B6 metabolism.
    Acta vitaminologica et enzymologica, 1975, Volume: 29, Issue:1-6

    Topics: Anticonvulsants; Aspartate Aminotransferases; Child; Child, Preschool; Epilepsy; Erythrocytes; Human

1975
B vitamins in epileptics.
    Bibliotheca nutritio et dieta, 1986, Issue:38

    Topics: Adult; Anticonvulsants; Biotin; Epilepsy; Erythrocytes; Female; Humans; Male; Pyridoxal Phosphate; P

1986
The concentration of homovanillic and 5-hydroxyindoleacetic acids in ventricular and lumbar CSF. Studies in patients with extrapyramidal disorders, epilepsy, and other diseases.
    Neurology, 1972, Volume: 22, Issue:11

    Topics: Adolescent; Adult; Basal Ganglia Diseases; Cerebral Palsy; Cerebral Ventricles; Diazepam; Dihydroxyp

1972
Tryptophan load tests and pyridoxal-5-phosphate levels in epileptic children. I. Non-progressive brain damage and degenerative brain disorders.
    Acta paediatrica Scandinavica, 1966, Volume: 55, Issue:4

    Topics: Adolescent; Brain Diseases; Child; Child, Preschool; Epilepsy; Female; Humans; Infant; Male; Pyridox

1966
Tryptophan load tests and pyridoxal-5-phosphate levels in epileptic children. II. Cryptogenic epilepsy.
    Acta paediatrica Scandinavica, 1966, Volume: 55, Issue:4

    Topics: Child; Child, Preschool; Epilepsy; Humans; Infant; Pyridoxal Phosphate; Pyridoxine; Tryptophan

1966